Diagnosis of Oral Diseases

2 PDQ ORAL DISEASE
White Lesions
Actinic (Solar) Cheilitis

Etiology
• Chronic, excessive exposure to solar radiation; ultraviolet
spectrum (ranging from 290 to 320 nm) most damaging
• Fair-complexioned people more severely affected than others
• May progress to cutaneous actinic keratosis and/or squamous
cell carcinoma
Clinical Presentation
• Vermilion portion of lower lip
• Pale irregularly opaque (keratotic) surface with intervening red
(atrophic) zones
• Obfuscated to effaced cutaneous-vermilion border
• More advanced lesions are scaly, crusted and/or indurated.
• Progression to carcinoma often heralded by persistent
ulceration or erosion
Microscopic Findings
• Hyperkeratosis
• Epithelial atrophy
• Variable degrees of epithelial dysplasia
• Amphophilic to basophilic change in submucosa (elastosis)
• Telangiectasia
Diagnosis
• Thermal/chemical burn ruled out by history
• Chronic ultraviolet light exposure
• Biopsy findings
Differential Diagnosis
• Exfoliative cheilitis
• Squamous cell carcinoma
White Lesions 3
Treatment
• Prevention of further damage with sunscreens blocking long-
wave ultraviolet A (UVA) and short-wave ultraviolet B
(UVB) light
• Biopsy of clinically suspicious areas
• CO2 laser vermilionectomy
• Topical 5-fluorouracil or vermilionectomy for severe disease
• Excision or resection-reconstruction if malignant
transformation has occurred
Prognosis
• Lifelong follow-up
• Up to 10% develop into squamous cell carcinoma.
• When carcinoma develops, growth tends to be slow and
metastasis occurs late; 85 to 90% long-term survival
4 PDQ ORAL DISEASE
Candidiasis
Etiology
• Infection with a fungal organism of the Candida species, usually
Candida albicans
• Associated with predisposing factors: most commonly,
immunosuppression, diabetes mellitus, antibiotic use, or xero-
stomia (due to lack of protective effects of saliva)
• Acute (thrush)
• Pseudomembranous
• Painful white plaques representing fungal colonies on
inflamed mucosa
• Erythematous (acute atrophic): painful red patches caused
by acute Candida overgrowth and subsequent stripping of
those colonies from mucosa
• Chronic
• Atrophic (erythematous): painful red patches; organism
difficult to identify by culture, smear, and biopsy
• “Denture-sore mouth”: a form of atrophic candidiasis
associated with poorly fitting dentures; mucosa is red and
painful on denture-bearing surface
• Median rhomboid glossitis: a form of hyperplastic
candidiasis seen on midline dorsum of tongue anterior to
circumvallate papillae
• Perlèche: chronic Candida infection of labial commissures;
often co-infected with Staphylococcus aureus
• Hyperplastic/chronic hyperplastic: a form of hyperkeratosis
in which Candida has been identified; usually buccal
mucosa near commissures; cause and effect not yet proven
• Syndrome associated: chronic candidiasis may be seen in
association with endocrinopathies
Diagnosis
• Microscopic evaluation of lesion smears
• Potassium hydroxide preparation to demonstrate hyphae
• Periodic acid–Schiff (PAS) stain
• Culture on proper medium (Sabouraud’s, corn meal, or
potato agar)
• Biopsy with PAS, Gomori’s methenamine silver (GMS), or
other fungal stain of microscopic sections
White Lesions 5
• Allergic or irritant contact stomatitis
• Atrophic lichen planus
Treatment
• Topical or systemic antifungal agents
• For immunocompromised patients: routine topical agents
after control of infection is achieved, usually with systemic
azole agents
• See “Therapeutics” section
• Correction of predisposing factor, if possible
• Some cases of chronic candidiasis may require prolonged
therapy (weeks to months).
Prognosis
• Excellent in the immunocompetent host
6 PDQ ORAL DISEASE
Exfoliative Cheilitis
Etiology
• Causes may be atopic, contact, factitious, infectious, systemic,
or medication induced.
• Usually involves lower lip (in both genders); can involve both
lips
• Tender or asymptomatic crusts and impacted scale of vermilion
• Minimal inflammation
Diagnosis
• Clinical appearance
• Nonspecific microscopy results
• Atopic cheilitis
• Actinic cheilitis
• Contact cheilitis
Treatment
• Determination of cause
• Supportive care
• Topical or intralesional corticosteroids, including lip ointments/
pomade (hypoallergenic)
• Topical tacrolimus ointment
Prognosis
• Chronic
• Psychologic support for factitial cheilitis
White Lesions 7
8 PDQ ORAL DISEASE
Fordyce’s Granules
Etiology
• Ectopic sebaceous glands within the oral mucosa and vermilion
portion of the lips
• Multiple, scattered, yellowish pink, maculopapular granules
• Buccal mucosa and vermilion of lips predominantly affected
• Asymptomatic
• Increasingly prominent after puberty
Diagnosis
• Bilateral distribution and appearance
• Lack of symptoms
• If biopsy performed, normal sebaceous glands in the absence of
hair follicles noted
• Candidiasis
Treatment
• None
• Reassurance
Prognosis
• Excellent
White Lesions 9
10 PDQ ORAL DISEASE
Geographic Tongue
Etiology
• Unknown; may be familial
• May be related to atopy
• Small percentage associated with cutaneous psoriasis
• May be symptomatic in association with spicy or acidic foods
• Focal red depapillated areas bordered by slightly elevated,
yellowish margin
• Dynamic behavior: changes in shape, size, intensity day to day
• Dorsal and lateral tongue surfaces affected predominantly
• Ventral tongue and other areas less often involved
• Often associated with fissured tongue
Diagnosis
• Location and appearance
• Biopsy confirmation usually unnecessary
• Reiter’s syndrome
• Lichen planus
• Lupus erythematosus
• Candidiasis
• Psoriasis
Treatment
• None, if asymptomatic
• Topical corticosteroids, if symptomatic
Prognosis
• Excellent
• No malignant potential
• May last months to years with periods of remission
White Lesions 11
12 PDQ ORAL DISEASE
Hairy Leukoplakia
Etiology
• Probably due to opportunistic Epstein-Barr virus (EBV) infec-
tion of epithelial cells
• Usually in an immunocompromised or immunosuppressed host
• Usually arises on lateral tongue border
• Early lesions are fine, white, vertical streaks with an overall
corrugated surface
• Later lesions may be thickened to be plaque-like
• Extensive lesions can involve dorsum of tongue and buccal
mucosa
• May serve as a pre-AIDS (acquired immunodeficiency syndrome)
sign
Diagnosis
• Incisional biopsy findings show characteristic EBV nuclear
inclusions in upper-level keratinocytes
• Frictional hyperkeratosis
• Lichen planus
• Hyperplastic candidiasis
Treatment
• None necessary; predisposing condition to be investigated
• Can be suppressed with acyclovir for esthetics
• Antiviral acyclovir
• Podophyllin resin topically
Prognosis
• May herald human immunodeficiency virus (HIV) disease in
vast majority of cases
• Also may be present after AIDS is established
White Lesions 13
14 PDQ ORAL DISEASE
Hairy Tongue
Etiology
• Generally unknown
• May be related to poor oral hygiene, soft diet, heavy smoking,
systemic or topical antibiotic therapy, radiation therapy, xero-
stomia, or use of oxygenating mouth rinses (H2O2, sodium
perborate)
• Elongated, hyperkeratotic filiform papillae on tongue dorsum
producing a “furred” to “hairy” texture
• Color varies from tan to brownish yellow to black depending
upon diet, drugs, chromogenic organisms
• Symptoms usually minimal; may produce gagging or tickling
sensation on palate
Diagnosis
• Clinical features
• Culture or cytologic studies not helpful
Treatment
• Physical débridement (brushing with a soft-bristled toothbrush,
5 to 15 strokes, once or twice daily)
• Topical podophyllin (5% in benzoin) followed by débridement
• Elimination of cause, if identified
Prognosis
• Excellent
White Lesions 15
16 PDQ ORAL DISEASE
Leukoedema
Etiology
• Unknown
• Benign; common in general population, with racial clustering
in Blacks
• Symmetric, asymptomatic
• Buccal mucosa involved by gray-white, diffuse, milky surface
with an opalescent quality
• Wrinkled surface features at rest
• Dissipation of changes with stretching of mucosa
Diagnosis
• Clinical recognition is sufficient.
• Biopsy findings will show marked intracellular edema of
spinous layer.
• Individual cells with clear cytoplasm and compact nuclei
• Normal basal cell layer
• Cheek chewing
• Hereditary benign intraepithelial dyskeratosis
• White sponge nevus
• Lichen planus
• Candidiasis
Treatment
• None necessary; no relation to dysplasia/carcinoma
• Reassurance
Prognosis
• Excellent
White Lesions 17
18 PDQ ORAL DISEASE
Leukoplakia
Etiology
• Essentially unknown, although many cases related to use of
tobacco or areca nut in its various formulations
• Other possible factors include nutritional deficiency (iron, vitamin
A) and infection (Candida albicans, human papillomavirus).
• An idiopathic white (sometimes white-and-red) patch
• Most common on lip, gingiva, buccal mucosa
• Increased risk of dysplasia or carcinoma when occurring on
tongue, floor of mouth, vermilion portion of lip
• Clinical subsets include homogeneous, verrucous, speckled,
and proliferative verrucous leukoplakia (proliferative form may
be multiple and persistent)
• Cases may advance or regress unpredictably—reflective of a
dynamic process
• Most occur in the fifth decade and beyond
• Progress to dysplasia or malignancy may occur with little or no
change in clinical appearance.
Diagnosis
• Performance of a biopsy is mandatory after elimination of any
suspected causative factors
• Multiple biopsies of large lesions are needed to be performed
due to microscopic heterogeneity within a single lesion.
• Other white lesions
• Frictional keratosis • Burn (thermal/chemical)
• Hyperplastic candidiasis • Lichen planus
• Genetic alterations (genodermatoses)
• White sponge nevus • Hereditary benign intra-
• Dyskeratosis epithelial dyskeratosis
Treatment
• Excision modalities (surgery, laser ablation, cryosurgery)
• Option to observe lesions diagnosed as benign hyperkeratosis
or mild dysplasia
White Lesions 19
• Possibly photodynamic therapy

• Topical cytotoxic drugs (bleomycin) remain experimental.
• Recurrences common following apparent complete excision
Prognosis
• Guarded
• Observation with repeat biopsies to be performed
Prevention
• Elimination of tobacco use and heavy alcohol consumption
• Recurrences may be reduced by systemic retinoid therapy.
• Possible dietary measures
20 PDQ ORAL DISEASE
Lichenoid Drug Eruptions

Etiology
• Hypersensitivity to drugs including sulfasalazine, angiotensin-
converting enzyme inhibitors, nonsteroidal anti-inflammatory
drugs, β-blockers, gold, antimalarials, sulfonylurea compounds
• Contact hypersensitivity
• Idiopathic reaction to dental restorations including amalgam,
composites, gold, other metals
• White striae or papules, as with lichen planus
• Lesions may appear ulcerative with associated tenderness or
pain.
• Most often in buccal mucosa and attached gingiva, but any site
may be involved
Diagnosis
• Identification and elimination of causative substance
• Biopsy of areas unresponsive to elimination strategy to demon-
strate characteristic keratosis and interface inflammation and
associated changes
• Patch testing performed to confirm contact allergens
• Lichen planus
• Leukoplakia
• Dysplasia/carcinoma
Treatment
• Alternative drugs or material to be chosen
• Topical corticosteroid applications
• Topical tacrolimus applications
Prognosis
• Good
• Observation while lesions exist
White Lesions 21
22 PDQ ORAL DISEASE
Lichen Planus
Etiology
• Unknown
• Autoimmune T cell–mediated disease targeting basal ker-
atinocytes (antigen unknown)
• Lichenoid changes associated with galvanism, graft-versus-host
disease (GVHD), certain drugs, contact allergens
• Up to 3 to 4% of population have oral lichen planus
• 0.5 to 1% of population have cutaneous lichen planus; 50%
also have oral lesions (25% with oral lesions have concomitant
skin lesions)
• White females (60%)
• Occurs in fourth to eighth decades
• Variants: reticular (most common oral form); erosive (painful);
atrophic, papular, plaque types; bullous (rare)
• Bilateral and often symmetric distribution
• Oral site frequency: buccal mucosa (most frequent), then
tongue, then gingiva, then lips (least frequent)
• Skin sites: forearm, shin, scalp, genitalia
• Hyperkeratosis
• Basal keratinocyte necrosis
• Lymphocytes at epithelial-connective tissue interface
Diagnosis
• Examination of oral mucosa, skin, genitalia
• Negative ocular mucosa history; no history of blistering
• Use of drugs, galvanism, GVHD to be ruled out
• Biopsy
• Direct immunofluorescence–fibrinogen and cytoid bodies at
interface help confirm
• Lichenoid drug eruptions • Erythema multiforme
• Lupus erythematosus • Contact stomatitis
• Mucous membrane pemphigoid
White Lesions 23
Treatment of Oral Lichen Planus

• Mild to moderate: topical corticosteroids
• Severe: systemic immunosuppression, chiefly with prednisone
• Corticosteroid-sparing drugs with prednisone
• Topical tacrolimus ointment
Prognosis
• Control, not cure, can be expected.
• Good prognosis; rare malignant transformation (0.5–3%)
• May be cyclic; may last for years/decades
• Tends to be chronic
24 PDQ ORAL DISEASE
Morsicatio Buccarum/Labiorum
(Cheek and Lip Chewing)
Etiology
• Chronic, low-grade biting habit
• Shaggy, white, keratotic surface
• Surface often appears granular to macerated
• More uniform keratotic surface may develop over time if habit
continues
• Most common sites are lip and buccal mucosa
• Very irregular, fimbriated surface keratin
• Surface bacterial colonization
• No connective tissue changes
Diagnosis
• Presentation
• Biopsy
• Leukoedema
• Leukoplakia
• Lichen planus
• Lichenoid tissue reactions
Treatment
• Elimination of hyperfunction habit
Prognosis
• Excellent
White Lesions 25
26 PDQ ORAL DISEASE
Proliferative Verrucous Leukoplakia

Etiology
• Some associated with human papillomavirus types 16 and 18
• Role of tobacco and other risk factors
• Represents a clinicopathologic spectrum of disease
• Multiple lesions develop from hyperkeratosis and/or verrucous
hyperplasia to verrucous carcinoma or papillary squamous cell
carcinoma
• Slowly progressive and persistent
• Initially a flat hyperkeratotic to warty surface
• Surface may be friable
• Typically multiple and recurrent
• Seen in middle-aged to elderly patients
Diagnosis
• Based upon appearance, clinical course, and microscopic
diagnosis (ie, clinical-pathologic correlation)
• Microscopic diagnoses include epithelial hyperplasia, hyper-
keratosis, verrucous hyperplasia, “atypical papillary-verrucal
proliferation,” verrucous or well-differentiated squamous cell
carcinoma
• Idiopathic leukoplakia
• Oral warts/condyloma
• Verrucous/squamous cell carcinoma
Treatment
• Surgical excision
• Mucosal stripping or excision for benign lesions
• Wide excision to resection for advanced lesions
• Laser ablation for benign/atypical lesions
• Systemic retinoids to control keratosis
White Lesions 27
Prognosis
• Progression to carcinoma frequently occurs, usually many
years after initial lesion(s) develops.
• Fair to good prognosis after malignant transformation
• Frequent follow-up visits recommended and surgical inter-
vention as new/recurrent lesions develop
28 PDQ ORAL DISEASE
Smokeless Tobacco Keratosis (Snuff Pouch)

Etiology
• Persistent habit of holding ground tobacco within the
mucobuccal vestibule
• Usually in men in Western countries
• Powdered snuff use prevalent in Southeast United States often
by women
• Mucosal pouch with soft, white, fissured appearance
• Surface may be pumice-like to verrucous
• Leathery surface due to chronic tobacco use over many years
• Hyperkeratosis with parakeratotic “chevron sign” at surface
• Increased vascularity
• Older lesions with hyalinization in submucosa and minor
salivary glands
• Epithelial dysplasia and carcinoma may evolve.
Diagnosis
• Biopsy
• Leukoplakia (idiopathic)
• Mucosal burn (chemical/thermal)
Treatment
• Discontinuation of habit
• If dysplasia is present, stripping of mucosal site
Prognosis
• Generally good with tobacco cessation
• Malignant transformation to squamous cell carcinoma or verru-
cous carcinoma occurs but less frequently than does smoking-
related carcinoma.
White Lesions 29
30 PDQ ORAL DISEASE
Submucous Fibrosis
Etiology
• Results from direct mucosal contact with a quid containing
areca (betel) nut, tobacco, and other ingredients; alkaloids and
tannin in the areca nut are liberated by action of slaked lime
within the quid, which is wrapped with the betel leaf
• Risk of oral squamous cell carcinoma is increased several-fold
• Early phase: tenderness, vesicles, erythema, burning, melanosis
• Later phase: mucosal rigidity, trismus
• Sites most often affected: buccal mucosa, soft palate
• Leukoplakia of surface with pallor
• Deep scarring, epithelial atrophy in cheeks, soft palate
• Biopsy results show submucosal deposition of dense collagen.
• Epithelial thinning, hyperkeratosis
• Epithelial dysplasia found in up to 15% of cases
Diagnosis
• Appearance
• History
• Lichen sclerosus
Treatment
• Intralesional corticosteroid placement
• Surgical release of scar bands in latter stages
• Careful follow-up and vigilance for development of squamous
cell carcinoma
Prognosis
• Irreversible
• Fair
White Lesions 31
Both photographs courtesy of Dr. John S. Greenspan.

32 PDQ ORAL DISEASE
White Sponge Nevus

Etiology
• Hereditary (autosomal-dominant) disorder of keratinization
affecting nonkeratinizing oral, esophageal, and anogenital
mucosal epithelium
• Point mutations in the keratin 4 and/or 13 genes
• Asymptomatic
• Deeply folded, thickened, white mucosa
• Buccal mucosa chiefly affected
• No functional impairment
• Increased prominence during second decade
• Parakeratosis, acanthosis, intracellular edema
• Perinuclear condensation of keratin
Diagnosis
• Family history
• Microscopic findings
• Idiopathic leukoplakia
• Chemical/thermal burn
• Chronic low-grade trauma (morsicatio)
Treatment
• None required
• No malignant potential
Prognosis
• Excellent
White Lesions 33
34 PDQ ORAL DISEASE
Red/Blue Lesions
Ecchymosis
Etiology
• Soft tissue hemorrhage
• Blood dyscrasia with secondary thrombocytopenia, hemophilia
• Vascular wall defects
• Coagulopathy
• Trauma
• Larger than pinpoint spots (ie, larger than petechiae)
• Nonvesicular, macular surface
• Lesions do not blanch with pressure
• Red to reddish blue to brown color
Diagnosis
• Characteristic size, color
• History
• Blood count, coagulation profile
• Hemophilia, Kaposi’s sarcoma, hemangioma, thrombocytopenia,
von Willebrand’s disease, leukemia, trauma
Treatment
• Identification of etiology, and corresponding treatment
Prognosis
• Excellent
Red/Blue Lesions 35
36 PDQ ORAL DISEASE
Erythroplakia
Etiology
• Unknown: a red patch that cannot be clinically attributed to
another condition
• Contributing factors include tobacco use, alcohol consumption
• Red, often velvety, well-defined patch(es)
• Most common on floor of mouth, retromolar trigone area,
lateral tongue
• Usually asymptomatic
• May be smooth to nodular
• Chiefly in males
Diagnosis
• Appearance; history of tobacco/alcohol use
• Biopsy results differentiate from inflammatory and atrophic
lesions
• Erythematous (atrophic) candidiasis
• Kaposi’s sarcoma
• Ecchymosis
• Contact stomatitis
• Vascular malformation
• Geographic tongue/erythema migrans
Treatment
• Surgical excision if proven dysplastic/malignant
Prognosis
• Fair to good depending upon microscopic diagnosis
• Almost all cases are premalignant to malignant upon initial
discovery.
Red/Blue Lesions 37
38 PDQ ORAL DISEASE
Fissured Tongue
Etiology
• Unknown
• May be hereditary
• Occurs with greater prevalence as population ages
• Multiple crenations or fissures
• May be seen in association with erythema migrans/geographic
tongue
• Prominence increases with age
• A component of Melkersson-Rosenthal syndrome
• May be a source of halitosis
Diagnosis
• Characteristic appearance
• If symptomatic (pain, burning), may be related to the following:
• Secondary candidiasis (antifungal prescription)
• Idiopathic factors
Treatment
• Usually none
• With candidal colonization, topical antifungal preparations are
effective.
• Careful débridement with soft-bristled brush, 5 to 15 strokes,
once or twice daily
Prognosis
• Excellent
Red/Blue Lesions 39
40 PDQ ORAL DISEASE
Hemangioma
Etiology
• Benign developmental anomalies of blood vessels that may
be subclassified as congenital hemangiomas and vascular
malformations
• “Congenital hemangioma” usually noted initially in infancy or
childhood (hamartomatous proliferation)
• Congenital hemangioma due to proliferation of endothelial cells
• “Vascular malformations” due to abnormal morphogenesis of
arterial and venous structures
• Congenital lesions usually arise around time of birth, grow
rapidly, and usually involute over several years.
• Malformations generally are persistent, grow with the child,
and do not involute.
• Color varies from red to blue depending on depth, degree of
congestion, and caliber of vessels
• Range in size from few millimeters to massive with disfigurement
• Most common on lips, tongue, buccal mucosa
• Sturge-Weber syndrome (trigeminal encephaloangiomatosis)
includes cutaneous vascular malformations (port wine stains)
along trigeminal nerve distribution, mental retardation, and
seizures.
Diagnosis
• Aspiration
• Blanching under pressure (diascopy)
• Imaging studies
• Purpura
• Telangiectasia
• Other vascular neoplasms
Red/Blue Lesions 41
Treatment
• Observation
• Congenital hemangiomas typically involute, whereas vascular
malformations persist.
• Surgery (scalpel, cryosurgery, laser [argon, copper])—congenital
hemangiomas usually are circumscribed and more easily
removed than are vascular malformations, which are poorly
defined. (Vascular malformations are associated with excessive
bleeding and recurrence.)
• Sclerotherapy
• Microembolization followed by resection for large malforma-
tions or if bleeding is problematic
Prognosis
• Guarded
42 PDQ ORAL DISEASE
Hereditary Hemorrhagic Telangiectasia

(Rendu-Osler-Weber Syndrome)
Etiology
• Not uncommon, familial (autosomal-dominant) mucocutaneous
vascular disease
• Some cases may be nonfamilial (spontaneous mutation).
• Arteriovenous (eg, pulmonary) malformations also can occur.
• Multifocal, macular to slightly papular red lesions of skin and
mucosa
• Most common on lips, tongue, buccal mucosa, finger tips
• Commonly associated with epistaxis due to involvement of
nasal mucosa
• Increase in number and prominence with age
• Blanch under pressure (diascopy positive)
• Lesions can affect gastrointestinal mucosa, which may rupture
with associated signs of chronic gastrointestinal blood loss;
may produce anemia
Diagnosis
• Family history
• Distribution of lesions
• CREST syndrome (calcinosis cutis, Raynaud’s phenomenon,
esophageal dysfunction, sclerodactyly, telangiectasia)
• Chronic hepatitis
• Radiation-induced vascular alterations
Treatment
• Observation
• Monitoring of pulmonary lesions; embolization if indicated
Prognosis
• Lifelong follow-up/monitoring
• 4 to 10% death rate from complications of the disease
Red/Blue Lesions 43
44 PDQ ORAL DISEASE
Kaposi’s Sarcoma
Etiology
• Several forms
• Classic idiopathic form affecting extremeties
• Endemic form (African)
• Immunosuppression-associated form
• Acquired immunodeficiency syndrome (AIDS)-associated form
• All forms, especially AIDS-associated and immunosuppression-
associated forms, may be caused by or closely related to a her-
pesvirus (human herpesvirus 8 [HHV-8] or Kaposi’s
sarcoma–associated herpesvirus [KSHV]).
• Classic form associated with slow but pernicious growth over
many years; oral lesions rarely seen
• Endemic form more rapid; oral lesions rarely seen
• AIDS-associated KS most commonly seen on keratinized
mucosa/mucoperiosteal tissues; strong predilection for hard
palate, followed by gingiva, buccal mucosa, and tongue (preva-
lence decreasing with treatment for AIDS)
• Evolution from bluish macule to nodule(s)
• Evolution to multiple lesions
• May precede or follow cutaneous lesions
Diagnosis
• May occur in up to one-third of AIDS patients
• Biopsy showing spindle cell proliferation with vascular slits,
extravascular red blood cells
• Hematoma
• Hemangioma
• Ecchymosis
• Malignant melanoma
• Pyogenic granuloma
• Amalgam tattoo
Red/Blue Lesions 45
Treatment of AIDS-Associated Form

• Radiation therapy: single fraction or equivalent fractionated
therapy of 800 cGy
• Intralesional therapy: interferon-α, vincristine, vinblastine
(2 mg/cc), sclerosing agents (sodium morrhuate)
• Systemic chemotherapy: interferon-α, vincristine, vinblastine,
bleomycin, daunorubicin
• Most treatment is palliatively directed.
Prognosis
• Variable, depending upon host’s immune status, but generally
poor in AIDS-associated form
46 PDQ ORAL DISEASE
Petechiae
Etiology
• Viral infection (Epstein-Barr virus [EBV]-mononucleosis;
measles), rickettsial infection
• Thrombocytopenia, leukemia
• Disseminated intravascular coagulation (DIC)
• Trauma: prolonged coughing, frequent vomiting, giving birth,
fellatio, violent Valsalva maneuvers
• Pinpoint hemorrhage into mucosa/submucosa
• Asymptomatic
• Usually involves the soft palate
• No blanching on pressure (diascopy)
Diagnosis
• History, determination of underlying cause
• See “Etiology”
Treatment
• None; observation only
Prognosis
• Variable, depending upon etiology
Red/Blue Lesions 47
48 PDQ ORAL DISEASE
Plasma Cell Gingivitis

Etiology
• Usually represents a hypersensitivity phenomenon to an agent
such as the following:
• Cinnamon/cinnamon flavoring
• Candy flavors
• Toothpaste/mouthwash
• Plaque antigens
• Reddened, velvety gingival surface
• Surface epithelium becomes nonkeratinized.
• Limited to attached gingiva
Diagnosis
• Response to elimination of possible etiologic agents
• Biopsy results show plasma cell infiltration within the
submucosa and lamina propria beneath an acanthotic
epithelium.
• Patch testing
• Wegener’s granulomatosis
• Chronic candidiasis
• Lichen planus
• Mucous membrane pemphigoid
Treatment
• Elimination of causative factor
Prognosis
• Reversal with removal of causative agent
Red/Blue Lesions 49
50 PDQ ORAL DISEASE
Pyogenic Granuloma
Etiology
• A reactive hyperplasia of capillaries and fibroblasts
• Related to chronic, persistent trauma or irritation (eg, calculus
or foreign body)
• Misnomer—neither pyogenic nor granulomatous
• Occurs at any age, but usually in children, young adults, and
women
• Red, lobular to smoothly contoured appearance
• When ulcerated, a yellow fibrinous exudate covers the lesion.
• Sessile to pedunculated commonly on gingiva, but also on
areas that are traumatized (eg, lower lip, buccal mucosa)
• Bleeds easily but is painless
• Hyperplastic granulation tissue
• Often lobular aggregation of proliferative vascular tissue
• Acute and chronic inflammation may be present, especially if
ulcerated
Diagnosis
• History of gradual to rapid onset
• Identification of a stimulus or causative factor (eg, trauma,
physical irritant)
• Histologic evaluation
• Peripheral giant cell granuloma
• Peripheral ossifying fibroma
• Metastatic tumor
Red/Blue Lesions 51
Treatment
• Local excision, scalpel excision
• Laser ablation
• Electrosurgery
• If on gingiva, excision should be extended to the periosteum or
periodontal ligament
Prognosis
• Excellent
• Recurrence occasional
52 PDQ ORAL DISEASE
Varices
Etiology
• An abnormal venous dilatation
• Congenital or from damage to vessel wall (trauma, ultraviolet
light)
• Occur with increasing frequency over 40 years of age
• Blue, lobulated surface
• Painless, evolves slowly
• Common on lower lip, sublingual regions
• Blanches with compression (diascopy)
• May become thrombosed
Diagnosis
• Histologic viewing of large-caliber, thin-walled vein
• Mucocele
• Vascular neoplasm
• Blue rubber bleb nevus syndrome
• Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber
syndrome)
Treatment
• Observation only, if stable
• Elimination by excision, sclerotherapy, or laser ablation
Prognosis
• Excellent
Red/Blue Lesions 53
54 PDQ ORAL DISEASE
Vesiculobullous Diseases
Epidermolysis Bullosa
Etiology
• A diverse group of predominantly cutaneous, but also mucosal,
mechanobullous diseases
• Inherited form: autosomal dominant or recessive patterns may
occur
• Acquired form (acquisita): autoimmune from autoantibodies
(immunoglobulin G [IgG]) to type VII collagen deposited with-
in the basement membrane zone and upper dermis or lamina
propria
• Variable, depending upon the specific form of many subtypes
recognized
• Mucosal lesions range in severity from mild to debilitating,
depending on subtype:
• Inherited forms have wide range of oral mucosal
involvement, with most severe form (autosomal recessive,
dermolytic) also demonstrating enamel hypoplasia and caries
• Acquisita form with mucous membrane pemphigoid variant
shows oral and conjunctival erosions/blisters
• Mucosal involvement absent in several variants
• Scarring and stricture formation common in severe recessive
forms
• Mucosa is often friable, but it may be severely blistered, erod-
ed, or ulcerated.
• Loss of oral anatomic landmarks may follow severe scarring
(eg, tongue mucosa may become smooth and atrophic with
episodes of blistering and scarring).
• Obliteration of vestibules, reduction of oral opening,
ankyloglossia
• Scarring can be associated with atrophy and leukoplakia, with
increased risk for squamous cell carcinoma development.
Vesiculobullous Diseases 55
• Bullae vary in location depending upon the form that is present:
• Intraepithelial in nonscarring forms
• At epithelial–connective tissue junction in dystrophic forms
• Subepithelial/intradermal in scarring forms
• Ultrastructural findings are as follows:
• Intraepithelial forms associated with defective cytokeratin
groups
• Junctional forms associated with defective anchoring
filaments at hemidesmosomal sites (epithelial–connective
tissue junction)
• Dermal types demonstrate anchoring fibril or collagen
destruction.
Diagnosis
• Distribution of lesions
• Family history
• Microscopic evaluation
• Ultrastructural evaluation
• Immunohistochemical evaluation of basement membrane zone
using specific labeled antibodies as markers for site of blister
formation
• Varies with specific form
• Generally includes the following:
• Bullous pemphigoid
• Mucous membrane (cicatricial) pemphigoid
• Erosive lichen planus
• Dermatitis herpetiformis
• Porphyria cutanea tarda
• Erythema multiforme
• Bullous impetigo
• Kindler syndrome
• Ritter’s disease
(continued)
56 PDQ ORAL DISEASE
Treatment
• Acquisita form:
• Some recent success with colchicine and dapsone
• Immunosuppressive agents including azathioprine,
methotrexate, and cyclosporine may be effective
• Acquisita and inherited forms:
• Avoidance of trauma
• Dental prevention strategies including extra-soft brushes,
daily topical fluoride applications, dietary counseling
Prognosis
• Widely variable depending on subtype
58 PDQ ORAL DISEASE
Erythema Multiforme
Etiology
• Many cases preceded by infection with herpes simplex; less
often with Mycoplasma pneumoniae or other organisms
• May be related to drug consumption, including sulfonamides,
other antibiotics, analgesics, phenolphthalein-containing laxa-
tives, barbiturates
• Another trigger may be radiation therapy.
• Essentially an immunologically mediated reactive process,
possibly related to circulating immune complexes
• Acute onset of multiple, painful, shallow ulcers and erosions
with irregular margins
• Early mucosal lesions are macular, erythematous, and occa-
sionally bullous.
• May affect oral mucosa and skin synchronously or
metachronously
• Lips most commonly affected with eroded, crusted, and
hemorrhagic lesions (serosanguinous exudate) known as
Stevens-Johnson syndrome when severe
• Predilection for young adults
• As many as one-half of oral cases have associated erythematous
to bullous skin lesions.
• Target or iris skin lesions may be noted over extremities.
• Genital and ocular lesions may occur.
• Usually self-limiting; 2- to 4-week course
• Recurrence is common.
Diagnosis
• Appearance
• Rapid onset
• Multiple site involvement in one-half of cases
• Biopsy results often helpful, but not always diagnostic
• Viral infection, in particular, acute herpetic gingivostomatitis
(Note: Erythema multiforme rarely affects the gingiva.)
• Pemphigus vulgaris
• Major aphthous ulcers
Treatment
• Mild (minor) form: symptomatic/supportive treatment with
adequate hydration, liquid diet, analgesics, topical cortico-
steroid agents
• Severe (major) form: systemic corticosteroids, parenteral fluid
replacement, antipyretics
• If evidence of an antecedent viral infection or trigger exists,
systemic antiviral drugs during the disease or as a prophylactic
measure may help.
• See “Therapeutics” section for details.
Prognosis
• Generally excellent
• Recurrences common
60 PDQ ORAL DISEASE
Hand-Foot-and-Mouth Disease
Etiology
• A very common enterovirus infection (coxsackievirus A10 or
A16), which may occur in mild epidemic proportion, chiefly in
children
• Incubation period is short, usually less than 1 week
• Oral mucosal lesions with focal herpes simplex–like appearance,
usually involving nonkeratinized tissue (soft palate, floor of
mouth, labial-buccal mucosa)
• Accompanying palmar, plantar, and digital lesions are deeply
seated, vesicular, and erythematous
• Short course with mild symptoms
Diagnosis
• Concomitant oral and cutaneous lesions
• Skin lesions commonly involve hands and feet.
• Skin lesions may involve buttocks.
• Antibody-titer increase measured between acute and recovery
phases
• Herpangina
• Herpes simplex infection
• Acute lymphonodular pharyngitis
Treatment
• Symptomatic treatment only
• Patient should be cautioned against the use of aspirin to
manage fever.
Prognosis
• Excellent
• Lifelong immunity, but it is strain specific
62 PDQ ORAL DISEASE
Herpangina
Etiology
• Most often by members of coxsackievirus group A (7, 9, 10,
and 16) or group B (1–5)
• Occasionally due to echovirus 9 or 17
• Incubation period of 5 to 9 days
• Acute onset
• Usually endemic in young children; usually occurs in summer
• Often subclinical
• Posterior oral cavity, tonsillar pillars involved
• Macular erythematous areas precede short-lived vesicular erup-
tion, followed by superficial ulceration
• Accompanied by pharyngitis, dysphagia, fever, malaise,
headache, lymphadenitis, and vomiting
• Self-limiting course, usually under 2 weeks
Diagnosis
• Other viral illnesses to be ruled out or separated
• Course, time of year, location of lesions, contact with known
infected individual
• Hand-foot-and-mouth disease
• Varicella
• Acute herpetic gingivostomatitis
Treatment
• Soft diet
• Hydration
• Antipyretics
• Chlorhexidine rinses
• Compounded mouth rinses
Prognosis
• Excellent
64 PDQ ORAL DISEASE
Herpetic Stomatitis: Primary

Etiology
• Herpes simplex virus (HSV)
• Over 95% of oral primary herpes due to HSV-1
• Physical contact is mode of transmission
• 88% of population experience subclinical infection or mild
transient symptoms
• Most cases occur in those between 0.5 and 5 years of age.
• Incubation period of up to 2 weeks
• Abrupt onset in those with low or absent antibody to HSV-1
• Fever, anorexia, lymphadenopathy, headache, in addition to
oral ulcers
• Coalescing, grouped, pinhead-sized vesicles that ulcerate
• Ulcers show a yellow, fibrinous base with an erythematous halo
• Both keratinized and nonkeratinized mucosa affected
• Gingival tissue with edema, intense erythema, pain, and
tenderness
• Lips, perioral skin may be involved
• 7- to 14-day course
Diagnosis
• Usually by clinical presentation and pattern of involvement
• Cytology preparation to demonstrate multinucleate virus-
infected giant epithelial cells
• Biopsy results of intact macular area show intraepithelial
vesicles or early virus-induced epithelial (cytopathic) changes
• Viral culture or polymerase chain reaction (PCR) examination
of blister fluid or scraping from base of erosion
• Herpangina
• Varicella
• Herpes zoster (shingles)
• Erythema multiforme (typically no gingival lesions)
Treatment
• Soft diet and hydration
• Antipyretics (avoid aspirin)
• Chlorhexidine rinses
• Systemic antiviral agents (acyclovir, valacyclovir) if early in
course or in immunocompromised patients
• Compounded mouth rinse
Prognosis
• Excellent in immunocompetent host
• Remission/latent phase in nearly all those affected who have
adequate antibody titers
66 PDQ ORAL DISEASE
Impetigo
Etiology
• Cutaneous bacterial infection: Streptococcus and
Staphylococcus species
• Is spread through direct contact
• Highly contagious
• Honey-colored, perioral crusts preceded by vesicles
• Flaccid bullae less common (bullous impetigo)
Diagnosis
• Culture of organism (usually group A, β-hemolytic streptococci
or group II Staphylococcus aureus)
• Herpes simplex (recurrent)
• Exfoliative cheilitis
• Drug eruptions
• Other vesiculobullous diseases
Treatment
• Topical antibiotics (mupirocin, clindamycin)
• Systemic antibiotics
Prognosis
• Excellent
• Rarely, poststreptococcal glomerulonephritis may develop.
68 PDQ ORAL DISEASE
Mucous Membrane Pemphigoid

Etiology
• Autoimmune; trigger unknown
• Autoantibodies directed against basement membrane zone
antigens
• Vesicles and bullae (short lived) followed by ulceration
• Multiple intraoral sites (occasionally gingiva only)
• Usually in older adults
• 2:1 female predilection
• Ocular lesions noted in one-third of cases
• Proclivity for scarring in ocular, laryngeal, nasopharyngeal,
and oropharyngeal tissues
• Subepithelial cleft formation
• Linear pattern IgG and complement 3 (C3) along basement
membrane zone; less commonly IgA
• Direct immunofluorescence examination positive in 80% of cases
• Indirect immunofluorescence examination usually negative
• Immunoreactants deposited in lamina lucida in most patients
Diagnosis
• Biopsy
• Direct immunofluorescent examination
• Epidermolysis bullosa acquisita
Treatment
• Topical corticosteroids
• Systemic prednisone, azathioprine, or cyclophosphamide
• Tetracycline/niacinamide
• Dapsone
Prognosis
• Morbidity related to mucosal scarring (oropharyngeal,
nasopharyngeal, laryngeal, ocular, genital)
• Management often difficult due to variable response to
corticosteroids
• Management often requires multiple specialists working in
concert (dental, dermatology, ophthalmology, otolaryngology)
70 PDQ ORAL DISEASE
Paraneoplastic Pemphigus
Etiology
• Autoimmune, triggered by malignant or benign tumors
• Autoantibodies directed against a variety of epidermal antigens
including desmogleins 3 and 1, desmoplakins I and II, and
other desmosomal antigens, as well as basement membrane
zone antigens
• Short-lived vesicles and bullae followed by erosion and ulcera-
tion; resembles oral pemphigus
• Multiple oral sites
• Severe hemorrhagic, crusted erosive cheilitis
• Painful lesions
• Cutaneous lesions are polymorphous; may resemble lichen
planus, erythema multiforme, or bullous pemphigoid
• Underlying neoplasms such as non-Hodgkin’s lymphoma,
leukemia, thymoma, spindle cell neoplasms, Waldenström’s
macroglobulinemia, and Castleman’s disease
• Suprabasilar acantholysis, keratinocyte necrosis, and vacuolar
interface inflammation
• Direct immunofluorescent testing is positive for epithelial cell
surface deposition of IgG and C3 and a lichenoid tissue reaction
interface deposition pattern
• Indirect immunofluorescent testing is positive for epithelial cell
surface IgG antibodies
• Special testing with mouse and rat bladder, cardiac muscle, and
liver may demonstrate paraneoplastic pemphigus antibodies
that bind to simple columnar and transitional epithelia
Diagnosis
• Biopsy of skin or mucosa
• Direct immunofluorescent examination of skin or mucosa
• Indirect immunofluorescent examination of sera including
special substrates
• Stevens-Johnson syndrome
• Erosive oral lichen planus
Treatment
• Identification of concurrent malignancy
• Immunosuppressive therapy
Prognosis
• Good with excision of benign neoplasms
• Grave, usually fatal, with malignancies
• Management is very challenging.
72 PDQ ORAL DISEASE
Pemphigus Vulgaris
Etiology
• An autoimmune disease where antibodies are directed toward
the desmosome-related proteins desmoglein 3 or desmoglein 1
• A drug-induced form exists with less specificity in terms of
immunologic features, clinical presentation, and histopathology
• Over 50% of cases develop oral lesions as the initial
manifestation
• Oral lesions develop in 70% of cases
• Painful, shallow irregular ulcers with friable adjacent mucosa
• Nonkeratinized sites (buccal, floor, ventral tongue) often are
initial sites affected
• Lateral shearing force on uninvolved skin or mucosa can
produce a surface slough or induce vesicle formation
(Nikolsky sign)
• Separation or clefting of suprabasal from basal layer of
epithelium
• Intact basal layer of surface epithelium
• Vesicle forms at site of epithelial split
• Nonadherent spinous cells float in blister fluid (Tzanck cells)
• Direct immunofluorescence examination positive in all cases
• IgG localization to intercellular spaces of epithelium
• C3 localization to intercellular spaces in 80% of cases
• IgA localization to intercellular spaces in 30% of cases
• Indirect immunofluorescence examination positive in 80%
of cases
• General correlation with severity of clinical disease
Diagnosis
• Mucosal manifestations
• Direct/indirect immunofluorescent studies
• Drug reaction
• Paraneoplastic pemphigus
Treatment
• Systemic immunosuppression
• Prednisone, azathioprine, mycophenolate mofetil,
cyclophosphamide
• Plasmapheresis plus immunosuppression
• IVIg for some recalcitrant cases
Prognosis
• Guarded
• Approximately a 5% mortality rate secondary to long-term
systemic corticosteroid–related complications
74 PDQ ORAL DISEASE
Recurrent Herpetic Stomatitis: Secondary

Etiology
• Herpes simplex virus
• Reactivation of latent virus
• Prodrome of tingling, burning, or pain at site of recurrence
• Multiple, grouped, fragile vesicles that ulcerate and coalesce
• Most common on vermilion border of lips or adjacent skin
• Intraoral recurrences characteristically on hard palate or
attached gingiva (masticatory mucosa)
• In immunocompromised patients, lesions may occur in any
oral site and are more severe (herpetic geometric glossitis).
Diagnosis
• Characteristic clinical presentation and history
• Viral culture or PCR examination of blister fluid or scraping
from base of erosion
• Cytologic smear
• Direct immunofluorescence examination of smear
• Herpangina
Treatment
• Acyclovir or valacyclovir early in prodrome
• Supportive
• Acyclovir may be used for prophylaxis for seropositive trans-
plant patients
• Ganciclovir may be used for human immunodeficiency virus
(HIV)-positive patients, especially those co-infected with
cytomegalovirus.
• For recurrent herpes labialis, see “Therapeutics” section.
Prognosis
• Excellent
• Healing without scarring within 10 to 14 days
• Protracted healing in HIV-positive patients
76 PDQ ORAL DISEASE
Stevens-Johnson Syndrome
Etiology
• A complex mucocutaneous disease affecting two or more
mucosal sites simultaneously
• Most common trigger: antecedent recurrent herpes simplex
infection
• Infection with Mycoplasma also may serve as a trigger.
• Medications may serve as initiators in some cases.
• Sometimes referred to as “erythema multiforme major”
• Labial vermilion and anterior portion of oral cavity usually
affected initially
• Early phase is macular followed by erosion, sloughing, and
painful ulceration
• Lip ulcers appear crusted and hemorrhagic.
• Pseudomembrane; foul-smelling presentation as bacterial
colonization supervenes
• Posterior oral cavity and oropharyngeal involvement leads to
odynophagia, sialorrhea, drooling
• Eye (conjunctival) involvement may occur.
• Genital involvement may occur.
• Cutaneous involvement may become bullous.
• Iris or target lesions are characteristic on skin.
• Subepithelial separation with basal cell liquefaction
• Intraepithelial neutrophils
• Epithelial and connective tissue edema
• Perivascular lymphocytic infiltrate
Diagnosis
• Usually made on clinical grounds
• Histopathology is not diagnostic.
• Paraneoplastic pemphigus
• Bullous pemphigoid
• Stomatitis medicamentosa
Treatment
• Hydration and local symptomatic measures
• Topical compounded oral rinses
• Systemic corticosteroid use controversial
• Recurrent, virally associated cases may be reduced in frequency
with use of daily, low-dose antiviral prophylactic therapy
(acyclovir, famciclovir, valacyclovir).
• May require admission to hospital burn unit
Prognosis
• Good; self-limiting usually
• Recurrences not uncommon
78 PDQ ORAL DISEASE
Varicella and Herpes Zoster

Etiology
• Primary and recurrent forms due to varicella-zoster virus (VZV)
• Primary VZV (chickenpox): a childhood exanthem
• Secondary (recurrent) VZV (herpes zoster/shingles) infection:
most common in elderly or immunocompromised adults
• Varicella (chickenpox)
• Fever, headache, malaise, and pharyngitis with a 2-week
incubation
• Skin with widespread vesicular eruption
• Oral mucosa with short-lived vesicles that rupture forming
shallow, defined ulcers
• Unilateral, dermatomal, grouped vesicular eruption of skin
and/or oral mucosa
• Vesicles may coalesce prior to ulceration and crusting.
• Lesions are painful.
• Prodromal symptoms along affected dermatome may occur.
• Pain, paresthesia, burning, tingling
• Postherpetic pain may be severe.
Diagnosis
• Clinical appearance and symptoms
• Cytologic smear with cytopathic effect present (multinucleated
giant cells)
• Viral culture or PCR examination of blister fluid or scraping
from base of erosion
• Serologic evaluation of VZV antibody
• Biopsy with direct fluorescent examination using fluorescein-
labeled VZV antibody
• Primary herpes simplex/acute herpetic gingivostomatitis
• Recurrent intraoral herpes simplex
Treatment
• Symptomatic management in primary infection
• Antiviral drugs (especially acyclovir) in immunocompromised
patients or patients with extensive disease
• Systemic corticosteroids may be used to help control/prevent
postherpetic neuralgia.
• Pain control to prevent “CNS imprinting”
Prognosis
• Generally good
• Recurrences more likely in immunosuppressed patients
80 PDQ ORAL DISEASE
Ulcerative Conditions
Actinomycosis
Etiology
• An infection caused by one of the Actinomyces group of
bacteria, chiefly the israelii species
• Most common site is the mandible, producing cervicofacial
disease
• Associated facial pain, paresthesia, low-grade fever, and
persistent swelling and discharge
• Bone lesions may be destructive in nature, with accompanying
rarefaction and/or sclerosis or periostitis.
• Cervicofacial form is usually insidious in onset, with a long-
term, low-grade course.
• Presents typically as a hard, chronic enlargement of the jaw;
extraoral abscess formation may be noted with drainage fluid
containing yellow sulfur granules (bacterial colonies)
Diagnosis
• Sulfur granules (1–4 mm) in exudate
• Peripheral club-like structures in bacterial colonies micro-
scopically
• Aerobic and anaerobic culture; actinomyces are anaerobic or
microaerophilic
• Infection: nocardiosis, fungal, staphylococcal, streptococcal
• Neoplasm (malignant)
Treatment
• Surgical débridement followed by prolonged antibiotic course
(penicillin is drug of choice)
• Surgical revision of extraoral drainage sites if indicated
Prognosis
• Excellent
Ulcerative Conditions 81
82 PDQ ORAL DISEASE
Acute Necrotizing Ulcerative Gingivitis

(Vincent’s Infection)
Etiology
• Fusobacterium nucleatum, Borrelia vincentii, and other
bacterial species including Prevotella and oral treponemes
• Infection requires modification of local or systemic factors
including immunosuppression, local hygiene, nutritional
deficiencies, intense smoking, and psychological stress.
• Engorged, enlarged, and blunted interdental papillae with cra-
teriform necrosis
• Symptoms include pain, regional lymphadenitis, fetid breath,
fever, and malaise.
• Ulcerated areas covered with grayish pseudomembrane
• Often accompanied by dental plaque and calculus
• Bleeding noted spontaneously or with minimal tissue
manipulation
• Extension of disease process into adjacent soft tissues noted
on occasion
Diagnosis
• Observation of characteristic blunted, necrotizing interdental
papillae with “punched-out” appearance
• Lesions on gingiva only
• Leukemia
• Immunosuppression-related conditions
• Primary herpetic gingivostomatitis
• Acute forms of leukemia
• Vesiculobullous mucosal diseases (mucous membrane
[cicatricial] pemphigoid, erosive/bullous lichen planus,
pemphigus vulgaris, paraneoplastic pemphigus)
Treatment
• Local débridement, ultrasonic scaling, good oral hygiene, and
home care
• Rinses of chlorhexidine, topical povidone-iodine

• Systemic antibiotics (tetracycline, metronidazole) may be
beneficial.
• Identification and elimination of predisposing factor(s)
• Underlying immunosuppression should be suspected if no
improvement noted
Prognosis
• Excellent
84 PDQ ORAL DISEASE
Aphthous Stomatitis
Etiology
• Unknown—probably represents a focal immunodysfunction;
no viral or other infectious agent identified
• Triggers vary from case to case (eg, increased stress/anxiety,
hormonal changes, dietary factors, trauma)
• Alterations in barrier permeability may be a factor, as occur with
human immunodeficiency virus/acquired immunodeficiency
syndrome (HIV/AIDS), bone marrow suppression, neutropenia,
gluten sensitivity, Crohn’s disease, ulcerative colitis, food allergy,
Behçet’s disease, and dietary deficiencies (iron, folate, vitamin
B12, zinc).
• Although likely immunologic in nature, the specific mechanism
is undetermined.
• Human leukocyte antigen (HLA) subtype susceptibility a
factor in some cases (-B12, -B51, and others)
• Affects 18 to 27% of the population; prevalence is approxi-
mately 20%
• Recurrent, self-limiting, painful ulcers
• Usually restricted to nonkeratinized oral and pharyngeal
mucosa (not hard palate or attached gingiva)
• Well-demarcated ulcers with yellow fibrinous base and
erythematous halo
• Three clinical forms: minor ulcers, major ulcers, herpetiform
lesions
• Minor variant (most common subtype)
• Occasional
• Single but more often multiple
• Less than 1 cm in diameter
• Oval to round shape
• Healing within 7 to 14 days
• Major variant (Sutton’s ulcers)
• 1 cm or greater in diameter
• Single or less commonly several
• Deep
• To ragged edges with elevated edematous margins
• May persist for several weeks to months

• Often heal with scarring
• Herpetiform variant (least common variant)
• Grouped superficial ulcers 1 to 2 mm in diameter; crops
of 10 to 100 lesions
• In nonkeratinized and keratinized tissues
• Healing within 7 to 14 days
• No etiologic role for herpes simplex virus
• Recurrent aphthous stomatitis occurs as simple (minor) aphthosis
(common) and complex (major) aphthosis (uncommon)
• Complex aphthosis (severe, almost continuous ulcerations;
disabling, large, or severe lesions)
• Simple aphthosis (mild; episodic: 1– 4 episodes/yr; few
lesions, usually minor or herpetiform)
• In AIDS patients, lesions are typically more severe and may
occur on any oral surface.
Diagnosis
• Usually has diagnostic clinical appearance of focal, well-
defined ulcers involving nonkeratinized mucosa
• History helpful; a recurrent process
• Positive family history
• Traumatic ulcer
• Chancre
• Recurrent intraoral herpes simplex stomatitis
• Cyclic neutropenia
Treatment
• Symptomatic therapy may be adequate.
• Systemic causative factors, if present, should be addressed.
• Tetracycline-based oral rinses may be helpful.
• Corticosteroid therapy is the most rational approach and is a
consistently effective treatment.
• Topical corticosteroids as gels, creams, or ointment 4 to
6 times/d to early lesions
• Intralesional corticosteroid injections
• Short-duration systemic corticosteroids (low to moderate
doses)
(continued)
86 PDQ ORAL DISEASE
• Other immunomodulating drugs may be helpful (dapsone,

hydroxychloroquine, topical tacrolimus, amelexanox).
• Colchicine (0.6–1.2 mg/d) is sometimes beneficial.
• Thalidomide treatment has shown efficacy in clinical trials.
Prognosis
• Simple aphthosis
• Excellent
• Cannot be cured
• Good control with corticosteroids is usually possible.
• Typically, severity decreases as patient ages.
• Complex aphthosis
• Needs medical evaluation for intercurrent disease
• Chronic problem
88 PDQ ORAL DISEASE
Behçet’s Disease
Etiology
• A multisystem disease secondary to an immunodysfunction
associated with certain HLA subtypes
• HLA-Bw51 clusters in those of Middle Eastern and Northern
Asian descent
• HLA-B12 noted more in those of European and North
American descent, with mucocutaneous presentation
• Classic signs noted in the oral cavity, eye, and genitalia
• Painful, sometimes debilitating, oral and genital aphthous ulcers
• Ocular lesions: painful conjunctivitis, uveitis, iritis, retinitis,
and hypopyon
• Cutaneous signs include the following:
• Erythema nodosum–like lesions
• Pustular folliculitis
• Thrombophlebitis
• Acneiform eruptions
• Positive pathergy sign is characteristic: sterile pustule at site of
sterile intradermal saline injection 48 hours prior
• Other systems, usually secondary to vasculitis, may be involved
in the following manner:
• Central nervous system (headache, paralysis,
meningoencephalitis)
• Gastrointestinal problems (diarrhea, inflammatory bowel
disease)
• Vascular thrombosis, hematologic and other organ system
manifestations
Diagnosis
• Oral aphthous ulcerations occurring at least three times per
year in association with characteristic manifestations within
other systems (ocular, genital, cutaneous problems)
• Reiter’s syndrome
• Crohn’s disease
• Vulvovaginal-gingival variant of erosive lichen planus
Treatment
• Systemic corticosteroids
• Immunosuppressive drugs (eg, interferon, TNFα inhibitors)
• Azathioprine, cyclosporine, chlorambucil, methotrexate
• Thalidomide has been proven helpful.
• Dapsone and colchicine may be of value in some cases.
Prognosis
• Chronic
• Manageable
90 PDQ ORAL DISEASE
Blastomycosis
Etiology
• Blastomyces dermatitidis produces the North American form of
this disease; Paracoccidioides brasiliensis causes South American
form and some endemic outbreaks in the United States.
• Transmission is usually by spore inhalation; most infections are
confined to the lungs. Extrapulmonary spread is hematogenous
to skin, mucosa, bone, viscera, meninges, and the genitouri-
nary tract.
• Acute: pneumonitis, fever, weight loss, night sweats, productive
cough
• Chronic: granulomatous lesions of oropharyngeal mucosa,
skin; pulmonary signs mimicking tuberculosis
• Skin and mucosal lesions are characterized by proliferative
verrucous growth, ulceration, and scarring. Mucosal lesions
may mimic carcinoma. Mucocutaneous disease indicates
disseminated disease.
Diagnosis
• Cytologic or histopathologic examination of tissue with identi-
fication of organism
• Culture of sputum or fresh biopsy material
• Potassium hydroxide preparation from lesion scraping
• Malignant tumor
• Tuberculosis
• Tertiary syphilis
Treatment
• Systemic antifungals: oral itraconazole
Prognosis
• Guarded
• Untreated disease slowly progressive, fatal
92 PDQ ORAL DISEASE
Crohn’s Disease
Etiology
• A granulomatous disease of unknown etiology
• Genetic factors coupled with environmental influences appear
of greatest importance
• Extraintestinal/oral findings may include the following:
• Nodular submucosal nodules of lips (granulomas)
• Polypoid masses with fissures and ulceration along the
buccal/labial sulcus
• Oral ulcers of nonspecific/aphthous type may develop.
• May present as orofacial granulomatosis such as
granulomatous cheilitis
• Lesions of pyostomatitis vegetans may be associated.
Diagnosis
• Correlation of mucosal lesions with intestinal symptoms of
cramping, diarrhea, and associated weight loss
• Oral mucosal biopsy results demonstrate noncaseating, epi-
theloid granulomas within submucosa
• Deep fungal diseases including blastomycosis
• Mycobacterial infections
• Tertiary syphilis and other treponemal infections
Treatment
• Management of underlying intestinal symptoms (nonsteroid
anti-inflammatory drugs, systemic corticosteroids)
• Local, oral mucosal lesions: monthly intralesional cortico-
steroid injections in areas of ulceration until improvement is
noted; treatment as needed
• Episodic burst of systemic corticosteroids in association with
local management of oral lesions; if condition persists, 7 to 10
days of prednisone with rapid taper to zero, with monitoring
• Management of any associated malabsorption may be helpful.

• Metronidazole, 5-aminosalicylic acid, ileal-release budesonide
Prognosis
• Related to response of intestinal symptoms to treatment
94 PDQ ORAL DISEASE
Histoplasmosis
Etiology
• A fungal infection caused by Histoplasma capsulatum
• Fungus endemic to Ohio and Mississippi River valleys
• Transmission: spore inhalation
• Oral lesions usually secondary to pulmonary lesions with
hematogenous dissemination
• General symptoms usually mild
• Oral lesions associated with disseminated form/prior
pulmonary lesions
• Chronic ulcerations with necrosis, elevated nodular margins
• May resemble squamous cell carcinoma
• On the tongue, a cobblestone ulcer may be noted.
• Oral ulcers persist until treatment of systemic disease.
Diagnosis
• Demonstration of organisms in biopsy specimen
• Culture
• Serologic demonstration of antigen or antibodies
• Tuberculosis
• Other deep fungal infections (eg, coccidioidomycosis, crypto-
coccosis, and blastomycosis)
• Chronic traumatic ulcer
• Tertiary syphilis
Treatment
• Sometimes none required
• Amphotericin B
• Ketoconazole, fluconazole, itraconazole
Prognosis
• May recover spontaneously
• Generally good unless immunosuppression present
96 PDQ ORAL DISEASE
Lupus Erythematosus
Etiology
• An autoimmune-/immunologically mediated condition
• Antibodies demonstrable against an array of cytoplasmic and
nuclear antigens
• Most often occurs in women
• Three forms are as follows:
• Chronic cutaneous (CCLE) or discoid (DLE)
• Subacute cutaneous (SCLE)
• Systemic (SLE)
• Black females have highest incidence
• Predominates in women over 40 years
• 80% of patients have concurrent cutaneous findings
• 30 to 40% of SLE patients have oral mucosal findings
• Oral mucosal lesions may appear lichenoid, keratotic, and
erosive.
• Labial vermilion with crusted, exfoliative, erythematous, and
keratotic appearance
• Oral findings are most common in CCLE or DLE.
Diagnosis
• Direct immunofluorescent examination of mucosal biopsy
• Serologic correlation (antinuclear antibodies: anti–SS-A, –SS-B,
and double-stranded deoxyribonucleic acid)
• Lichen planus
• Candidiasis
• Hypersensitivity/lichenoid eruption
• Leukoplakia
Treatment
• Complex—dependent on LE variant present and level of disease
expression
• Systemic corticosteroids and immunosuppressive agents for SLE
• Topical corticosteroid agents for intraoral lesions
• Low-dose hydroxychloroquine
Prognosis
• Good prognosis with CCLE or DLE form
• Variable prognosis with SLE
• SCLE has an intermediate prognosis between that of SLE and
CCLE or DLE forms.
98 PDQ ORAL DISEASE
Mucormycosis (Zygomycosis)
Etiology
• Organisms of Zygomycetes class: Rhizopus, Absidia, Mucor
genera
• Noted chiefly in immunosuppressed individuals and in uncon-
trolled diabetics
• Large, irregular, necrotizing ulcers
• Most often involves the palate with concomitant paranasal
sinus involvement
Radiographic Findings
• Maxillary sinus opacification
• Irregular sinus wall destruction
• Tissue necrosis with fungal invasion into blood vessels
• Nonseptate, branching, broad hyphae
Diagnosis
• Radiographic findings
• Maxillary sinus neoplasia
• Maxillary sinus aspergillosis
• Soft tissue infarction
• Soft tissue radionecrosis
• Other deep fungal infections
Treatment
• Surgical débridement
• Intravenous antifungal agents (amphotericin B, ketoconazole)
• Control of underlying disease process
Prognosis
• Good, depending upon underlying systemic factors
Photograph courtesy of Dr. John Knapp.

100 PDQ ORAL DISEASE
Neutropenic Ulcer
Etiology
• Idiopathic or iatrogenic neutropenia
• Usually noted when neutrophil count falls below 1,500/mm3
• Sharply defined ulcer(s), often with minimal peripheral erythema
• Ulcer base covered by fibrinous exudate
• Wide variation in size of ulcers
Diagnosis
• Clinical appearance correlated with results of peripheral blood
count
• Major aphthous ulcer
• Traumatic ulcer
• Necrotizing sialometaplasia
Treatment
• Identification and management of underlying neutropenia
• Supportive therapy
Prognosis
• Relative to ability to manage underlying neutropenia
Radiation-Induced Mucositis
Etiology
• Local tumoricidal doses of ionizing radiation
• Destruction of germinative layers of oral mucosal epithelium
within radiation portal
• May be enhanced by intraoral gram-negative bacteria
• Mucosal erythema, atrophy, necrosis, ulceration, and
pseudomembrane formation
• Generalized pain and dysfunction
• Ultimately, broadly based, contiguous ulcers form.
• Usually begins within 7 to 10 days following the start of
treatment
• Exacerbated by radiation-induced xerostomia or chemotherapy/
cytoreductive therapy
Diagnosis
• History and appearance
• Chemotherapy-induced stomatotoxicity
• Acute erythematous candidiasis
• Neutropenic ulcer
Treatment
• Systemic antiviral, antibacterial therapy
• Topical agents
• Water-soluble polymer films
• Antimicrobials
• Saline rinses
• Antifungal agents
• Granulocyte-macrophage colony-stimulating factor
• Local and hygiene measures
Prognosis
• Good
• Improves slowly subsequent to treatment
Squamous Cell Carcinoma

Etiology
• Majority (approximately 80%) related to tobacco and alcohol
abuse
• Some cases may be virus associated (human papillomavirus
types 16 and 18)
• Stepwise progression of genetic alterations now defined from
normal to dysplasia to carcinoma
• Early, usually a white or red-white focal surface alteration
• Later stages with ulceration, induration, elevated margins
• Most common sites: lateral tongue, floor of mouth
• Lower lip vermilion surface also a common location
• Advanced-stage disease has associated limitation of movement,
trismus, cervical lymph node metastases
• May erode or invade adjacent bone in later stages
• Irregular, destructive, ill-defined margins in later stages
• Usually well differentiated to moderately differentiated
• Invasive islands, cords of epithelial cells
• Individual cells with nuclear pleomorphism, increased nuclear-
to-cytoplasmic ratio, dyskeratosis
• Architectural disorganization of proliferating cells
Diagnosis
• Microscopic analysis of tissue specimen (biopsy)
• Chronic traumatic ulcer
• Primary syphilis
• Deep fungal infection
• Palatal necrotizing sialometaplasia
• Keratoacanthoma (labial)
Treatment
• Surgical excision is the treatment of choice.
• Combined surgery and radiation therapy for more advanced-
stage lesions
• Adjuvant chemotherapy plays a role in advanced disease.
Prognosis
• Results are stage related as follows:
• Stages 1 and 2: generally good prognosis
• Stages 3 and 4: generally fair to poor prognosis
Syphilis
Etiology
• Treponema pallidum spirochete
• Four clinical types (primary, secondary, and tertiary stages;
congenital form) plus neonatal form
• Primary stage (oral)
• Initial sign usually a firm nodule/papule
• Labial ulceration most common presentation
• Ulcer firm, indurated, painless
• Intraoral chancre is an ulcer covered by a pseudomembrane
• Lesion is highly infectious
• Regional cervical lymphadenopathy
• Spontaneous resolution
• Secondary stage
• Evolves after 6 weeks to 6 months if patient is untreated
• Reddish brown macular rash periorally; generalized
cutaneous rash
• Oral lesions are split papule at lip commissures, irregular
lesions, serpiginous ulcers, or erosions
• Mucous patches orally (ulcers covered by mucoid exudate)
• Lymphadenopathy
• Highly infectious
• Spontaneous resolution
• Tertiary stage
• Develops over 3 to 10 years after primary infection if untreated
or inadequately treated (more rapidly in immunocompromised
patients)
• Glossitis: atrophic, leukoplakia features
• Gumma: destructive, painless, solitary granulomatous ulcer;
midline of tongue, palate, especially
• Congenital form
• Hutchinson’s triad including mulberry molars, barrel-shaped
incisors
Diagnosis
• Clinical history, appearance
• Direct smear in primary- and secondary-stage lesions (dark field)
• Serologic studies (eg, VDRL [Venereal Disease Research

Laboratories] test): positivity noted in last phase of primary stage
• Biopsy
• Traumatic ulcer
• Leukoplakia
• Midline granuloma/Wegener’s granulomatosis
Treatment
• Antibiotics
• Parenteral penicillin (penicillin G benzathine) or ceftriaxone
• Oral tetracycline or doxycycline
Prognosis
• Excellent in primary and secondary stages
• Fair in late (tertiary) phase
Traumatic Granuloma
(Traumatic Eosinophilic Ulcer)
Etiology
• A benign, self-limiting, reactive process of oral mucosa of
unknown origin
• Some cases with no history of antecedent trauma
• Rapid onset
• Painful, indurated, crateriform ulcer
• Several weeks mean duration
• 60% occur on the tongue (lateral/ventral)
• Average diameter 1 to 2 cm
• Crateriform ulcer with fibrinous surface
• Deeper areas with granulation tissue, endothelial proliferation
• Inflammatory infiltrate with prominent macrophages
• Underlying muscle injury present with eosinophilic infiltrate
Diagnosis
• History and appearance
• Biopsy results/microscopic findings
Differential Diagnosis: Clinical

• Major aphthous ulcer
• Lymphoma
• Syphilis
• Granulomatous disease
• Sarcoidosis
• Wegener’s granulomatosis
• Tuberculosis
Differential Diagnosis: Microscopic

• Lymphoma
• Angiolymphoid hyperplasia with stromal eosinophilia
Treatment
• Excision
• Observation only
• Topical or intralesional corticosteroids
Prognosis
• Healing usually within 10 days if excised
• Most lesions heal after a few to several weeks without recur-
rence.
• Rare cases have multiple recurrences.
Traumatic Ulcer
Etiology
• Accidental/functional or factitious injury
• Tender to very painful
• Ulcer with yellow, fibrinous center and well-defined margins
• Inflammatory/erythematous periphery
• Fibrinous surface
• Mixed inflammatory infiltrate
• Granulation tissue at base of lesion
Diagnosis
• History of trauma
• Evaluation for ill-fitting dental prosthesis or orthodontic
appliance
• Nonspecific histologic findings
• Aphthous ulcer
• Neutropenia-related ulcer
• Factitial ulcer
• Primary syphilis (chancre)
Treatment
• None except elimination of cause
• Healing within 10 days
Prognosis
• Excellent
Tuberculosis
Etiology
• Mycobacterium tuberculosis usually; less commonly M. avium-
intracellulare
• Oral lesions form in relation to extension of disease beyond
the pulmonary focus
• Increased incidence in immunocompromised patients
• Chronic, nonhealing ulcer with induration
• Borders may be raised or rolled.
• Intrabony lesions are lytic and sequestrate with radiographic
features of osteomyelitis.
• Centrally necrotic granulomas with peripheral multinucleated
Langhans’ giant cells
• Positive Fite or Ziehl-Neelsen tissue staining of microorganisms
Diagnosis
• Clinical appearance and lesion persistence
• Histopathology
• Tuberculin skin test; two-step Mantoux test
• Culture
• Syphilis
• Deep mycotic infection
• Traumatic eosinophilic ulcer
• Lymphoma
Treatment
• Systemic chemotherapy: isoniazid, rifampin, streptomycin, and
others
• Note: Multidrug-resistant organism may be present.
Prognosis
• Good
• In the immunosuppressed patient, prognosis is fair.
Wegener’s Granulomatosis
Etiology
• Unknown
• A necrotizing vasculitis with preferential involvement of the
respiratory tract early in its course
• Oral involvement unusual and characterized by ulceration and
tissue destruction
• Jaw pain, gingival inflammation with petechiae or hyperplasia,
palatal ulceration with possible perforation
• Painful salivary gland enlargement may be encountered.
• Classic triad of upper airway, lung, and kidney involvement
not required for diagnosis
• May remain localized for prolonged periods prior to multi-
organ involvement
• Vasculitis, necrosis, and granulomatous inflammation
Diagnosis
• Oral or upper airway biopsy is helpful in less than one-half of
cases.
• Tissue biopsy for microscopic features
• Laboratory studies show the following:
• Antineutrophil cytoplasmic antibodies (ANCAs)—two stain-
ing patterns: cytoplasmic ANCA (high specificity) and peri-
nuclear ANCA
• Mild, normocytic, normochromic anemia (in 50%)
• Elevated erythrocyte sedimentation rate
• Lymphoma, including midline granuloma
• Late-stage syphilis
• Tuberculosis
Treatment
• Cyclophosphamide/prednisone
• Trimethoprim/sulfamethoxazole (as monotherapy or in
combination with immunosuppressive therapy)
Prognosis
• Fair
• Secondary complications related to long-term immuno-
suppression
Pigmentary Disorders
Addison’s Disease
Etiology
• Adrenal cortical atrophy—85% idiopathic (?autoimmune)
• Oral manifestations due to secondary melanocyte stimulation
by increased levels of adrenocorticotropic hormone (ACTH) or
β-lipotropin
• Brown macular pigmentation of local or diffuse quality
• Pigmentation usually seen in association with cutaneous
bronzing, weakness, weight loss, salt craving, nausea, vomit-
ing, hypotension
Diagnosis
• Confirmation of hypoadrenocorticism by plasma ACTH levels
after challenge/stimulation
• Biopsy of mucosa shows melanosis
• Smoker’s melanosis
• Physiologic/ethnic pigmentation
• Heavy metal deposition/argyrosis
• Medication-related pigmentation
• Peutz-Jeghers syndrome
Treatment
• Management of underlying adrenal insufficiency by cortico-
steroid replacement therapy
Prognosis
• Good with replacement therapy
Pigmentary Disorders 117
Amalgam Tattoo
Etiology
• Implantation or passive/frictional transfer of dental silver
amalgam into mucosa
• Gray to black focal macules, usually well defined, but may be
diffuse with no associated signs of inflammation
• Typically in attached gingiva, alveolar mucosa, buccal mucosa
• Occasionally may be visible radiographically
Diagnosis
• Radiographs may be useful (intraoral film placement)
• Biopsy may be necessary if clinical diagnosis is in doubt or to
rule out lesions of melanocytic origin
• Mucosal nevus
• Melanoma
• Mucosal melanotic macule
• Melanoacanthoma
Treatment
• Biopsy or observation only
Prognosis
• Little clinical significance if untreated
Melanoacanthoma
Etiology
• A reactive and reversible alteration of oral mucosal
melanocytes and keratinocytes
• Usually associated with local trauma
• Unilateral dark plaque; rarely multiple, bilateral
• Most often noted among Blacks and other non-Caucasians
• Occurs more often in women than men by a ratio of 3:1
• History of trauma and local irritation
• Forms rapidly, most often on buccal/labial mucosa
• Asymptomatic melanotic pigmentation
Diagnosis
• Clinical history of rapid onset
• Histologic evaluation
• Scattered dendritic melanocytes within spongiotic and
acanthotic epithelium
• Increased number of melanocytes along basal layer as single
units
• Melanoma
• Drug-induced pigmentation
• Mucosal nevus
• Amalgam tattoo
Treatment
• None after establishing the diagnosis
• Often resolves spontaneously
Prognosis
• Excellent
Mucosal Malignant Melanoma

Etiology
• Unknown
• Cutaneous malignant melanoma with relation to sun exposure
or familial-dysplastic melanocytic lesions
• Rare in oral cavity (< 1% of all melanomas) and sinonasal tract
• Most cases occur in those older than 30 years of age.
• Usually arises on maxillary gingiva and hard palate
• May exhibit early in situ phase: a macular, pigmented patch
with irregular borders
• Progression to deeply pigmented, nodular quality with ulceration
• May arise de novo as a pigmented or amelanotic nodule
• Rarely may be metastatic to the oral cavity as a nodular, usually
pigmented mass
• Early stage: atypical melanocytes at epithelial–connective tissue
interface, occasionally with intraepithelial spread
• Later infiltration into lamina propria and muscle
• Strict correlation to cutaneous malignant melanoma is not well
established, although, as in skin, a similar horizontal or in situ
growth phase often precedes the vertical invasive phase.
• Amelanotic forms may require use of immunohistochemical
identification: S-100 protein, HMB-45, Melan-A expression
Diagnosis
• Biopsy
• High index of suspicion
• Mucosal nevus
• Extrinsic pigmentation
• Melanoacanthoma
• Amalgam tattoo
Treatment
• Marginal parameters related to depth of invasion and pres-
ence of lateral growth
• Wide surgical margins; resection (including maxillectomy)
for large, deeper lesions
• Neck dissection in cases of deep invasion (< 1.25 mm)
Prognosis
• Generally poor for most oral malignant melanomas
• Less than 20% survival at 5 years in most studies
Mucosal Melanotic Macule and Ephelides

Etiology
• Most idiopathic, some postinflammatory, some drug-induced
• Multiple lesions suggest syndrome association, as follows:
• Peutz-Jeghers syndrome
• Laugier-Hunziker phenomenon
• Carney’s syndrome
• LEOPARD syndrome
• Most in adulthood (fourth decade and beyond)
• Most are solitary and well circumscribed
• Lower lip vermilion border most common site, mostly in
young women (labial melanotic macule)
• Buccal mucosa, palate, and attached gingiva also involved
(mucosal melanotic macule)
• Usually brown, uniformly pigmented, round to ovoid shape
with slightly irregular border
• Usually < 5 mm in diameter
• Normal melanocyte density and morphology
• Increased melanin in basal cells and subjacent macrophages
(mucosal melanotic macule)
• Increased melanin in basal cells with elongated rete pegs
(ephelides)
Diagnosis
• Biopsy
• Melanoacanthoma
• Congenital syndromes (Carney’s, Peutz-Jeghers, LEOPARD,
Laugier-Hunziker)
Treatment
• Observation
• Biopsy for esthetics
• If increase in size or development of atypical signs occurs,
macule should be removed to rule out malignant melanoma,
particularly if on palate or alveolar mucosa.
Prognosis
• Excellent
Mucosal Pigmentation: Extrinsic

(Drug or Metal Induced)
Etiology
• Occupational exposure—metals vapors (lead, mercury)
• Therapeutic—metal salt deposits (bismuth, cis-platinum, silver,
gold); also nonmetal agents, such as chloroquine, minocycline,
zidovudine, chlorpromazine, phenolphthalein, clofazimine, and
others
• Focal to diffuse areas of pigmentary change
• If heavy metals are the cause, a typical gray to black color is
seen along the gingival margin or areas of inflammation.
• Palatal changes characteristic with antimalarial drugs and
minocycline
• Most medications cause color alteration of buccal-labial
mucosa and attached gingiva.
• Darkened alveolar bone with minocycline therapy (10% at
1 year, 20% at 4 years of therapy)
Diagnosis
• History of exposure to, or ingestion of, heavy metals or drugs
• Differentiation from melanocyte-related pigmentation by
biopsy if necessary
• When localized: amalgam tattoo, mucosal melanotic macule,
melanoacanthoma, mucosal nevus, ephelides, Kaposi’s sarco-
ma, purpura, malignant melanoma, ecchymosis
• When generalized: ethnic pigmentation, Addison’s disease
• If asymmetric, in situ melanoma must be ruled out by biopsy.
Treatment
• Investigation of cause and elimination if possible
Prognosis
• Excellent
Nevus
Etiology
• Unknown
• Lesion of melanocytic origin within mucosa and skin
• Usually elevated, symmetric papule
• Pigmentation usually uniformly distributed
• Common on skin; unusual intraorally
• Palate and gingiva most often involved
• Most are intramucosal (“dermal”)
• Blue nevi are deeply situated and are composed of spindled
nevus cells.
• Other variants are rare; junctional and compound nevi
(no dysplastic nevi occur orally)
• Nevus cells are oval/round and are found in unencapsulated
nests (theques).
• Melanin production is variable.
Diagnosis
• Biopsy
• Melanoma
• Varix
• Amalgam tattoo/foreign body
• Ecchymosis
• Melanoacanthoma
Treatment
• Excision of all pigmented oral lesions to rule out malignant
melanoma is advised.
• Malignant transformation of oral nevi probably does not occur.
Prognosis
• Excellent
Nevus of Ota
Etiology
• Idiopathic/congenital
• A proliferation of dermal melanocytes over a specific anatomic
distribution
• Macular, grayish blue discoloration of skin and mucosa over
the distribution of the ophthalmic and maxillary branches of
the trigeminal nerve
• Unilateral distribution
• Sclera on the involved side may be affected.
• Diffuse unencapsulated proliferation of spindle-shaped
melanocytes within dermis/submucosa, parallel to surface
• Pigment production may be florid.
Diagnosis
• Clinical presentation
Treatment
• None
• Cosmetic
Prognosis
• Excellent
Pigmentation Disorders: Drug Induced

Etiology
• Therapeutic drug-related tissue pigmentation
• Many drugs may cause change—see listing below
• Macular mucosal discoloration (brown, gray, black)
• Palate and gingiva are most common sites affected
• In addition to mucosal changes, teeth in adults and children
may be bluish gray owing to minocycline/tetracycline use
(see “Tetracycline Staining” on page 138).
• Most cases are due to increased melanin production. Some are
related to the deposition of a drug complex or a metabolized
drug.
Diagnosis
• History
• Physiologic changes
Treatment
• Drug withdrawal
Prognosis
• Good
Drugs Capable of Producing Tissue Pigmentation

• Antimalarials: chloroquine, mepacrine, quinidine, old-time
antimalarials
• Antibiotics: tetracycline group, minocycline
• Antivirals: azidothymidine
• Phenothiazine: chlorpromazine
• Clofazimine
• Heavy metals: gold, mercury salts, silver nitrate, bismuth, lead
• Hormones: ACTH, oral contraceptives
• Cancer/chemotherapy drugs: busulfan, cyclophosphamide,
cis-platinum
• Other: methyldopa
Pigmentation Disorders: Physiologic

Etiology
• Normal melanocyte activity
• Seen in all ages
• Symmetric distribution over many sites, gingiva most commonly
• Surface architecture, texture unchanged
Diagnosis
• History
• Distribution
• Smoking-associated melanosis
• Superficial malignant melanoma
Treatment
• None
Prognosis
• Excellent
Pigmentation Disorders: Smoker’s Melanosis

Etiology
• Melanin pigmentation of oral mucosa in heavy smokers
• May occur in up to 1 of 5 smokers, especially females
taking birth control pills or hormone replacement
• Melanocytes stimulated by a component in tobacco smoke
• Brownish discoloration of alveolar and attached labial gingiva,
buccal mucosa
• Pigmentation is diffuse and uniformly distributed; symmetric
gingival pigmentation occurs most often.
• Degree of pigmentation is positively influenced by female
hormones (birth control pills, hormone replacement therapy).
• Increased melanin in basal cell layer
• Increased melanin production by normal numbers of
melanocytes
• Melanin incontinence
Diagnosis
• History of chronic, heavy smoking
• Biopsy
• Physiologic pigmentation
• Addison’s disease
• Medication-related pigmentation (drug-induced pigmentation by
chloroquine, clofazimine, mepacrine, chlorpromazine, quinidine,
or zidovudine)
• Malignant melanoma
Treatment
• None
• Reversible, if smoking is discontinued
Prognosis
• Good, with smoking cessation
Tetracycline Staining
Etiology
• Prolonged ingestion of tetracycline or its congeners during
tooth development
• Less commonly, tetracycline ingestion causes staining after
tooth formation is complete: reparative (secondary) dentin
cementum may be stained.
• Yellowish to gray (oxidized tetracycline) color of enamel and
dentin
• May be generalized or horizontally banded depending on
duration of tetracycline exposure
• Alveolar bone may also be stained bluish red (particularly
with minocyline use, 10% after 1 year and 20% after 4 years
of therapy).
Diagnosis
• Clinical appearance and history
• Fluorescence of teeth may be noted with ultraviolet illumination.
• Dentinogenesis imperfecta
Treatment
• Restorative/cosmetic dental techniques
Prognosis
• Good
Verrucal-Papillary Lesions
Condyloma Acuminatum
Etiology
• A sexually transmitted disease
• Associated with human papillomavirus (HPV) types 6, 11, 16,
and 18 most often
• Can result in autoinoculation of other sites via trauma
• Lesions located at the site of contact/traumatic event
• Usually on nonkeratinized tissues in immunocompetent
patients (soft palate, lingual frenum)
• Pink to whitish pink, exophytic papillary growths with pedun-
culated outline
• May be solitary or multiple and variably sized, up to 2 to 3 cm
• Can present as papillomatosis of upper respiratory tract
Diagnosis
• Demonstration of koilocytotic cellular changes on biopsy
• In situ hybridization or polymerase chain reaction reveals spe-
cific HPV subtype
• Electron microscopy demonstrates intranuclear virions
• Focal epithelial hyperplasia
• Multiple intraoral verruca vulgaris
• Squamous papilloma
Treatment
• Conservative removal
• Conventional surgery
• Laser ablation
• Topical podophyllin
Verrucal-Papillary Lesions 141
Prognosis
• Recurrences common
• Contagiousness and autoinoculation are considerations.
Focal Epithelial Hyperplasia

Etiology
• A viral infection (HPV 13 or 32), usually found in childhood
• Familial/ethnic clustering often noted, probably secondary
through horizontal viral transmission
• Often occurs in native Americans
• Numerous, slightly raised whitish pink asymptomatic papules
and irregular plaques that may become confluent
• Size of lesions ranges from a few millimeters to coalescent
papules several centimeters in dimension
• Well-defined acanthotic features
• Broadened, anastomosing epithelial ridges with occasional
superficial koilocytotic changes
Diagnosis
• Multiple, characteristic lesions
• Biopsy findings
• In situ deoxyribonucleic acid hybridization to demonstrate
HPV subtype
• Ultrastructural localization of intranuclear virions
• Condyloma acuminatum
• Multiple verruca vulgaris
Treatment
• None; lesions usually regress spontaneously
• Excision if esthetic needs demand
• Intralesional interferon therapy
Prognosis
• Excellent
• No reported malignant transformation
Keratoacanthoma
Etiology
• Unknown, may be related to several factors, as follows:
• Viral—HPV subtypes 11, 13, 24, 33, 57
• Altered expression of cell cycle proteins including cyclin E,
p53, PCNA
• Keratinocyte dedifferentiation reflected in deficient
desmoglein production
• Immunosuppression
• Sun damage
• May represent a highly differentiated form of squamous cell
carcinoma
• May indicate underlying alimentary neoplasia (Muir-Torre
syndrome)
• Usually solitary on sun-exposed areas, including lip
• Initially erythematous papule
• Rapid growth over 4 to 8 weeks
• Nodular, hemispheric, firm nodule
• Central keratin core
• Occasionally regresses spontaneously
• Extremely rare intraorally
Diagnosis
• Clinical evaluation, follow-up
• Histopathology shows keratin plus normal, peripheral epider-
mis and mature, premature keratinization; no invasion below
adnexa; marked pseudoepitheliomatous hyperplasia
• Molluscum contagiosum
• Warty dyskeratoma
• Verruca vulgaris
• Pilomatricoma
Treatment
• Observation and careful follow-up
• Local excision
• Cryotherapy
• Intralesional chemotherapy (methotrexate, 5-fluorouracil, or
bleomycin)
Prognosis
• Excellent
Lymphangioma
Etiology
• A benign proliferation of lymphatic vasculature
• Usually congenital in nature
• Superficial or deep in location
• Typically waxes and wanes in size
• Most commonly involves the tongue followed by lips, buccal
mucosa, palate
• Facial asymmetry may be a presenting sign.
• Superficial mucosal lymphangiomas resemble caviar or frog’s
eggs.
• Deeper lesions present as painless fluctuant masses such as
macroglossia.
• Often combined with blood vessels
• Rare variant may occur bilaterally on mandibular alveolar
ridge of neonates
Diagnosis
• Biopsy
• Lymphangiography
• Neurofibroma (deep)
• Hemihypertrophy syndromes
Treatment
• Excision
• If large lesions are stable, observation
• Sclerotherapy
Prognosis
• Variable, depending upon depth and extent of lesion
• Cavernous variant has guarded prognosis
Papillary Hyperplasia (Palatal Papillomatosis)

Etiology
• Generally attributed to ill-fitting maxillary denture
• Often associated with 24 h/d denture wearing
• Candida albicans overgrowth common
• May be noted in habitual mouth breathers (nondenture wearers)
• Erythematous palatal vault beneath denture
• Nodular papillary excrescences
• Generally asymptomatic
Diagnosis
• Biopsy results show fibrous and epithelial papillary hyperpla-
sia; may note pseudoepitheliomatous hyperplasia
• Chronic candidiasis
• Denture stomatitis
Treatment
• Establishment of good oral hygiene
• Possible antifungal therapy
• Surgical removal of affected mucosa, if excessive tissue hyper-
plasia
• Relining/remaking of denture
Prognosis
• Excellent
Pyostomatitis Vegetans
Etiology
• A pustular eruption usually associated with inflammatory
bowel disease and skin disease
• Liver dysfunction (sclerosing cholangitis) may be associated in
some cases.
• Mucosal pustules, erythema, edema
• Erosions and ulcers may form with serpiginous outlines (“snail
tracks”).
• Folds of nodular to hyperplastic tissue (“cobblestoning”)
• Neutrophilic and eosinophilic infiltrate into epithelium produc-
ing microabscesses
• Infiltration between epithelial clefts
• Epithelial hyperplasia
Diagnosis
• Correlation with underlying gastrointestinal disease, such as
the following:
• Ulcerative colitis
• Sclerosing cholangitis
• Malabsorption syndrome
• Oral Crohn’s disease
• Pseudomembranous (acute) candidiasis
• Melkersson-Rosenthal syndrome
• Orofacial granulomatosis
• Acanthosis nigricans
Treatment
• Successful management of underlying gastrointestinal disease
• Local anti-inflammatory agents
• Dapsone or sulfapyridine systemically
Prognosis
• Correlates with that of systemic disease
Squamous Papilloma
Etiology
• A benign epithelial proliferation
• HPV is found in most cases; several subtypes have been
identified, especially HPV 6 and 11.
• Exophytic, papillary mass, measuring less than 1 cm
• Usually pedunculated and soft in texture
• White
• Usually solitary; may be multiple
• Favors soft palate; uvula, tongue, gingiva, buccal mucosa may
also be involved
• Epithelial hyperplasia with fibrovascular cores
• Papillary projections may be sharp to blunt.
• Epithelium may be dysplastic in some lesions from human
immunodeficiency virus–positive patients.
Diagnosis
• Biopsy features
• Verruca vulgaris
• Verrucous carcinoma
Treatment
Prognosis
• Low recurrence rate
Verruca Vulgaris (Oral Warts)

Etiology
• Infection of mucosal epithelium by members of the human
papillomavirus group—usually HPV 2, 4, 6, or 11
• Papular to nodular and exophytic appearance
• Surface texture is cauliflower-like or verruciform in nature
• Perioral skin lesions may be brownish.
• Oral mucosal lesions are usually white to pink.
• May be pedunculated or broad based
• Intraoral sites of predilection include the lips, palate, and
attached gingiva.
• Multiple oral lesions may be evident in immunocompromised
patients.
• Surface hyperkeratosis
• Granulosis
• Koilocytosis
• Acquired immunodeficiency syndrome–associated oral warts
may appear dysplastic microscopically.
Diagnosis
• Immunohistochemical demonstration of HPV common antigen
• Keratoacanthoma
• Papillary squamous carcinoma
Treatment
• Excision
• Laser surgery
• Cryosurgery
• Electrosurgery
Prognosis
• Excellent in immunocompetent host
• Recurrence not uncommon
Verrucous Carcinoma
Etiology
• A well-differentiated, exophytic and endophytic squamous cell
carcinoma often associated with tobacco use, especially smoke-
less tobacco
• A primary or ancillary role for HPV is suspected.
• May be preceded by keratotic patch (see “Verrucous
Hyperplasia” on page 158)
• One-half of cases involve the buccal mucosa.
• Attached gingiva is involved in one-third of cases.
• Early, superficial lesions often are interpreted as verrucous
hyperplasia; lesions become exophytic, irregular, and indurated.
• Advancing lesions push into adjacent tissues.
• Late lesions invade the periosteum and destroy bone.
• Metastases are rare.
• Well-differentiated, blunt masses of epithelium extending into
submucosa
• Intense lymphocytic infiltrate adjacent to invasive front
Diagnosis
• Full-thickness specimen is necessary to establish diagnosis
• Verrucous hyperplasia
• Papillary squamous cell carcinoma
• Proliferative verrucous leukoplakia
Treatment
• Wide excision
• Radiation therapy may be effective.
• Dedifferentiation may occur spontaneously or after radiation
therapy.
Prognosis
• Excellent
• Local recurrence is a distinct possibility.
Verrucous Hyperplasia
Etiology
• Unknown; tobacco (smokeless) associated most commonly
• Role of HPV is unclear.
• A possible precursor to verrucous carcinoma
• Exophytic, papillary, keratotic fronds of epithelium
• May be part of the proliferative verrucous leukoplakia spectrum
• Papillary to verruciform surface projections
• Keratin varies in thickness
• Broad, bosselated epithelial ridges
• Well-differentiated cellular features
• Some similarity to early verrucous carcinoma
Diagnosis
• Microscopic features
• Verrucous carcinoma
• Papillary squamous cell carcinoma
• Proliferative verrucous leukoplakia
Treatment
• Excision or ablation (eg, laser, electrocautery)
• Continued observation
Prognosis
• Good with complete excision
• Recurrence is common.
Connective Tissue Lesions
Cementoblastoma
Etiology
• An uncommon, benign tumor of mesenchymal odontogenic
origin
• Unknown stimulus
• Usually affects patients under the age of 30 years
• Mandibular molar-premolar region is most common site
• Jaw expansion, pain, or tenderness
• Radiopacity with peripheral radiolucent halo
• Mass fused to root of affected tooth
• Focal radioactivity
• Obscuration of root apex
• Thin radiolucent rim
Diagnosis
• History of pain/tenderness
• Histologically: abundant number of cementoblasts are associated
with an irregular network of hard tissue (cementum)
• Microscopically similar to osteoid osteoma
• Focal osseous dysplasia
• Ossifying fibroma
• Osteoma
Treatment
• Enucleation (with associated tooth)
Prognosis
• Excellent
Connective Tissue Lesions 161
Cheilitis Glandularis
Etiology
• Unknown; may be familial in some cases
• Alternative etiologies include infectious, actinic, atopic, and
factitious origins
• Usually involves lower lip of adult males (may occasionally
involve both lips)
• Tender eversion and enlargement of lip
• Mucoid to purulent secretion at minor salivary gland orifices
Diagnosis
• Microscopy shows nonspecific inflammation.
• Biopsy results may show labial salivary gland hyperplasia and
ductal ectasia.
• Sialadenitis
• Granulomatous cheilitis (orofacial granulomatosis)
• Atopic cheilitis
• Actinic or photosensitivity cheilitis
Treatment
• Suppressive therapy with broad-spectrum antibiotics
Prognosis
• Chronic
• Good
Fibroma: Traumatic
Etiology
• Chronic low-grade trauma/irritation
• A reactive (hyperplastic), rather than neoplastic, process
• Firm
• Same as or more pale than surrounding tissue
• Pedunculated or broadly based (sessile) fibrous mass with a
smooth, elevated surface
• Asymptomatic and slow growing
• May be secondarily ulcerated or keratotic
• Typically in regions accessible to trauma: buccal mucosa, later-
al tongue margin, lower lip
Diagnosis
• Appearance
• Location
• Other submucosa/connective soft tissue tumors, as follow:
• Granular cell tumor
• Neurofibroma
• Lipoma
• Schwannoma
• Salivary tumor
Treatment
• Conservative local excision
Prognosis
• Excellent
Fibrous Dysplasia
Etiology
• Unknown
• A dysplastic process or developmental lesion of bone
• An asymptomatic tumor-like mass or deformity of bones
• May be monostotic (80%) or polyostotic (20%)
• Uncommonly, the polyostotic form may occur with endocrine
hyperfunction and focal cutaneous pigmentation (McCune-
Albright syndrome).
• Slow-growing, painless swelling of affected bone(s)
• Mandibular body most common site
• Facial asymmetry a frequent presenting sign
• Tooth displacement and malocclusion common
• Maxillary jaw lesions may be accompanied by involvement of
the zygoma, sphenoid, and, less commonly, the occiput (the
craniofacial variant).
• Ill-defined, uniformly radiopaque enlargement with “ground-
glass” qualities and diffuse, blended margins
• Mandibular lesions may show loculation.
• The craniofacial form may show skull base thickening.
• Intraoral films may show lamina dura obscurity.
Diagnosis
• Radiographic qualities
• Biopsy showing typical irregular trabeculae of woven bone
(“Chinese characters”) and fibroblastic, vascularized stroma
• Chronic sclerosing osteomyelitis
• Localized Paget’s disease
• Osteosarcoma
• Cemento-osseous dysplasias
Treatment
• Cosmetic recontouring, if necessary
• Observation only, if lesions are minimally developed and stable
Prognosis
• Most stabilize in adult life
• Some relapse noted in up to one-half of surgically recontoured
cases
• Malignant transformation rare unless previously irradiated
Fibrous Hyperplasia: Denture-Related

(Epulis Fissurata)
Etiology
• Trauma resulting from an ill-fitting denture
• Exuberant fibrous tissue repair secondary to repeated inflam-
mation and trauma
• Found on vestibular mucosa, usually at facial aspect of denture
flange
• Rounded folds of broadly based fibrous tissue surrounding the
overextended denture flange
• Ulceration often noted at depth of tissue folds
• More common in anterior segment of the jaws
• May occur on hard palate as a polypoid or leaf-like mass
Diagnosis
• Location and presence of a chronically ill-fitting denture
• Lymphoma
• Soft tissue tumor
Treatment
• Excision of all tissue
• Relining or reconstruction of new dentures after excision
Prognosis
• No recurrence anticipated with properly fitting denture
Florid Osseous Dysplasia

(Florid Cemento-osseous Dysplasia)
Etiology
• Unknown
• Strong predilection for middle-aged to elderly black females
• Bilateral and symmetric mandible involvement
• Body and posterior segment of mandible chiefly involved
• Early stage is mostly a radiolucent process.
• Later stages have multiple, mixed, radiolucent to radiopaque
nodularities.
• Purely lucent areas representing simple bone cysts may be seen
in conjunction with opacities.
Diagnosis
• Radiographic appearance
• Involved teeth are vital.
• Chronic, diffusing, sclerosing osteomyelitis
• Fibrous dysplasia
• Osteosarcoma
• Paget’s disease
Treatment
• In uncomplicated, asymptomatic cases, observation only
• If symptomatic, surgical removal of calcified masses with con-
comitant antibiotic coverage
• Bone saucerization and open packing may hasten progress.
Prognosis
• Good
• When infected, osteomyelitis-related morbidity occurs.
Gardner’s Syndrome
Etiology
• Autosomal-dominant condition
• Association of multiple osteomas, colonic and rectal polyposis,
cutaneous and mesenteric fibromas, epidermoid cysts of skin
• Gene is located on chromosome 5q
• Early clinical indicators may be osteomas of jaws and facial
bones.
• Colorectal polyps usually develop after osteomas.
• Facial asymmetry
• Multiple impacted teeth and odontomas not uncommon
• Well-defined, sclerotic, opaque masses
• Endosteal or periosteal origin
• Impacted and supplementary teeth are usually noted.
• Multiple odontomas may be noted.
Diagnosis
• Family history
• Multiple jaw bone, facial bone osteomas
• Concomitant polyposis of colon
• Exostoses of mandible/maxilla
Treatment
• Osteoma treatment is elective/cosmetic.
• Prophylactic colectomy as all patients ultimately develop colon
adenocarcinomas
• Genetic counseling
Prognosis
• Relates to colon adenocarcinoma development and behavior
Giant Cell Granuloma

Etiology
• Probably reactive or responsive in nature
• Speculation suggests it may represent a developmental anomaly.
• Bony expansion
• Most cases arise in those less than 30 years of age
• Female predominance
• Near exclusivity in mandible or maxilla; rarely in facial bones
• Occurrence in mandible predominates 3:1 over that in maxilla.
• Usually anterior to molar teeth
• Most cases are nonaggressive, slow growing, and asymptomatic,
with no cortical breakthrough or root end resorption.
• Some cases are recurrent and exhibit aggressive behavior with
pain, perforation, and rapid enlargement.
• No radiographic or histologic features can be used to separate
nonaggressive lesions from aggressive lesions.
• Usually multilocular, occasionally unilocular, radiolucency
• Margins are usually well defined; borders may be scalloped.
• Can displace teeth; less commonly it resorbs tooth roots
• Wide size variation at time of presentation
Diagnosis
• Incisional biopsy
• Primary hyperparathyroidism should be ruled out.
• Odontogenic lesions
• Ameloblastoma
• Odontogenic myxoma
• Odontogenic keratocyst
• Nonodontogenic lesions
• Hemangioma
• Aneurysmal bone cyst
• Traumatic bone cyst
• Hyperparathyroidism
• Giant cell tumor
Treatment
• Thorough curettage
• Marginal resection, if aggressive or recurrent
• Calcitonin may be successful in some cases.
• Intralesional corticosteroid placement in small lesions may be
successful.
Prognosis
• Aggressive variant has high recurrence rate
• Generally good
Gingival Hyperplasia: Generalized

Etiology
• May be nonspecific and reactive to local factors (plaque, calculus)
• May be related to hormonal changes (pregnancy, puberty)
• May be associated with drug use, including phenytoin,
cyclosporine, calcium channel blockers (especially nifedipine)
• A familial form exists
• Some cases may be related to Cowden disease and syndromes
such as Zimmerman-Laband, Rutherfurd’s, Cross, or Murray-
Puretic-Drescher.
• May be secondary to leukemic infiltrate
• Bulky enlargement of free and attached gingiva
• Blunted interdental papillae
• Soft and boggy to firm and dense in texture
• Pink to reddish blue
Diagnosis
• Medical history
• Biopsy
• See “Etiology.”
Treatment
• Identification and elimination of cause, if possible
• Gingivoplasty and improvement of oral hygiene may be indi-
cated in some cases.
Prognosis
• Good
• Often requires repeated excision
Granular Cell Tumor (Granular Cell Myoblastoma)

Etiology
• A benign neoplasm, probably derived from a Schwann cell pre-
cursor
• Most common site is the tongue
• Wide age range
• Nontender, asymptomatic submucosal mass, covered with
intact epithelium
• Usually dome-shaped, sessile mass; rarely pedunculated
Diagnosis
• Biopsy
• Traumatic fibroma
• Salivary gland tumor
• Neurofibroma
• Other soft tissue neoplasm (eg, rhabdomyoma, lipoma)
Treatment
• Excision
Prognosis
• Recurrence is rare.
• Rarely multiple tumors
Leukemia
Etiology
• Unknown
• Probably multifactorial: genomic instability including genetic
predisposition, syndromic association, prior chemotherapy,
environmental factors
• Oral expression most common with myelomonocytic and mye-
locytic forms, followed by lymphocytic form
• Petechiae, ecchymosis
• Spontaneous gingival hemorrhage
• Mucosal ulceration (neutropenic ulcers)
• Cervical lymphadenopathy
• Loose teeth (due to infiltration of periodontal ligament)
• Osteolytic lesions in up to one-half of childhood cases
• Expanded, coarse marrow spaces and trabeculae
• Alveolar bone destruction
• Loss of lamina dura and border of developmental dental crypts
• Periosteal reaction with “onion skin” effect
Diagnosis
• Peripheral blood analysis
• Bone marrow biopsy analysis
• Medication-induced gingival hyperplasia
• Idiopathic thrombocytopenia
• Lymphoma
Treatment
• Chemotherapy regimen(s)—these vary with form of disease
• Bone marrow transplantation
Prognosis
• Varies with form of disease and response to treatment
Lingual Bone Defect (Stafne Bone Cyst;

Static Bone Cyst)
Etiology
• Developmental depression of the lingual side of the mandible
• The aberrant lobe of the submandibular salivary gland and/or
adipose tissue fills the body of mandible defect. The depression
created produces characteristic radiographic findings.
• No symptoms
• Discovered incidentally
• Round to ovoid radiolucency below inferior alveolar canal,
above inferior border, and below third molar area
• Well defined by a dense hypercorticated margin
• Size range of 1 to 3 cm
• Rarely noted in premolar and canine areas
Diagnosis
Treatment
• None; recognition only
Prognosis
• Excellent
Lingual Thyroid
Etiology
• Failure of thyroid primordium to descend from foramen cecum
into anterior neck
• Midline mass at foramen cecum area
• Dark, well vascularized
• May interfere with swallowing and breathing in infancy
• Bleeding may occur.
Diagnosis
• Clinical appearance, location
• Demonstration of activity with radionuclide scan (technetium
99m)
• Confirmation of presence of cervical thyroid
• Thyroglossal duct cyst
• Lymphoma
Treatment
• Removal if functional thyroid is present in usual location
• Move/transplant to alternative site if other thyroid tissue does
not exist
Prognosis
• Excellent
Lipoma
Etiology
• A benign neoplasm of adipose cells
• Uncommon in the oral cavity
• Asymptomatic, slow-growing; usually circumscribed, sessile, or
pedunculated
• Soft and compressive with doughy consistency
• Most common sites include buccal mucosa, tongue, floor of
mouth
• May be deep seated with no color alteration
• Yellowish, lobulated quality when superficially located
• Surface of larger lesions often is covered by telangiectatic vessels.
Diagnosis
• Mature fat cells in lobular pattern
• Usually well circumscribed by thin fibrous capsule
• Several microscopic variations including infiltrating, pleomor-
phic, angioid, myxoid, and spindle cell types
• Other soft tissue tumor
• Minor salivary gland neoplasm
• Metastatic disease
Treatment
• Excision
Prognosis
• Recurrence rare with exception of infiltrating and intramuscu-
lar types
Macroglossia
Etiology
• Macroglossia is a clinical sign caused by one of the following
many conditions:
• Angioedema/allergic reaction
• Infection/abscess formation
• Inflammation related to trauma
• Granulomatous disease (sarcoidosis, tuberculosis, deep
fungal infections)
• Congenital disease (muscle hypertrophy or lymphangioma)
• Metabolic alteration (amyloidosis)
• Endocrine-related condition (acromegaly)
• Neoplasia/hamartomatous condition (neurofibromatosis)
• Usually diffuse enlargement; infectious forms may be asymmet-
ric and focal
• Transient forms often arise quickly.
• Persistent forms evolve slowly, may cause splaying of teeth
and scalloping of tongue borders, and may result in functional
problems (speaking, swallowing, deglutition)
Diagnosis
• Biopsy
Treatment
• Management is related to etiology and extent of involvement
• Surgical reduction in certain cases
Prognosis
• Related to etiology; from excellent to fair
Masseteric Hypertrophy
Etiology
• Usually secondary to hyperfunction (habitual)
• May be related to dystrophic or metabolic disease of muscle
• May be idiopathic
• Usually bilateral masseter muscle enlargement
• Painless, symmetric, evenly contoured
• Emphasized when muscle is contracted (ie, jaw is clenched)
• Responds to functional or hyperfunctional demands
Diagnosis
• History
• Muscle biopsy if metabolic disease suspected
• Sialoadenosis
• Parotid gland enlargement
• Bacterial infection
• Viral infection (mumps, ? cytomegalovirus)
• Autoimmune condition (Sjögren’s syndrome)
• Neoplastic infiltration
Treatment
• Dependent upon etiology
• Usually relates to defining cause and includes the following:
• Observation
• Management of underlying cause when appropriate
Prognosis
• Excellent
Melkersson-Rosenthal Syndrome
Etiology
• A chronic, idiopathic condition
• Part of orofacial granulomatosis spectrum that includes
Crohn’s disease, sarcoidosis, cheilitis granulomatosa
• Classic triad
• Chronic intermittent swelling of lip(s) or oral tissues
• Fissured tongue
• Facial nerve palsy (20%)
• Occasionally manifests as painless labial swelling (granuloma-
tous cheilitis/Miescher’s granuloma)
• May be seen in association with intestinal Crohn’s disease
• Lobulated, thickened mucosa of cheeks and tongue may be seen.
• Gingival surface granularity may be present uncommonly.
• “Correctable” causes must be excluded before an idiopathic
cause is accepted.
Diagnosis
• Biopsy
• Key feature is noncaseating epithelioid granulomas with multi-
nucleated giant cells
• Patch testing for contact allergy
• Angioedema
• Sarcoidosis
• Cellulitis/erysipelas of lip
Treatment
• If necessary, intralesional corticosteroid injections
• Tapering dose of systemic corticosteroids
• Clofazimine in slowly tapering dosage
• Dapsone and other nonsteroidal anti-inflammatory drugs
Prognosis
• Generally good
• May be persistent
Mucosal Neuroma
Etiology
• A component of the multiple endocrine neoplasia syndrome
type III (MEN III), (also called type 2b)
• Syndrome is related to proliferation of neural crest derivatives,
as follows:
• Medullary carcinoma of thyroid
• Pheochromocytoma
• Mucosal neuromas
• Ganglioneuromatosis of the bowel
• Autosomal-dominant transmission
• Gene is located on chromosome 10
Clinical Presentation of MEN III

• Thickened, prominent lips and frenula
• Conjunctival neuromas, corneal nerves
• Oral mucosal neuromas: tongue, lips, cheeks, commissures
• Marfanoid facies and habitus
• Medullary thyroid carcinoma
• Oral mucosal neuromas may be initial sign of syndrome
Laboratory Findings of MEN III

• Increased serum calcitonin levels
• Increased urinary vanillylmandelic acid
Microscopic Findings of Mucosal Neuroma

• Plexiform bundles of neural tissue
• Axons within bundles
Diagnosis
• Confirmation of neuroma presence
• Demonstration of increased serum calcitonin
• Neurofibromatosis
Treatment
• Thyroidectomy
• Follow-up for pheochromocytoma development
Prognosis
• Guarded, with 100% risk of medullary carcinoma
• 50% risk of pheochromocytoma
Neurofibroma
Etiology
• A benign neoplasm of peripheral nerve
• Concomitant proliferation of perineural fibroblasts and
Schwann cells
• Multiple lesions suggest neurofibromatosis syndrome.
• Syndromic form associated with autosomal-dominant inheri-
tance pattern due to mutation of NF1 or NF2 genes
• Tongue, buccal mucosa, mucobuccal fold most common sites
• Soft tissue findings: discrete nodules or diffuse lobular lesions
• Skin lesions with syndromic forms: café-au-lait macules,
(characteristically six or more); uniform pigmentation with
smoothly contoured borders
Radiographic Findings (When Intrabony Lesions Are Present)

• “Blunderbuss” expansion of inferior alveolar foramen
• Uniformly expanded alveolar canal in body of mandible
• Usually unencapsulated mass of spindle cells with gently wavy
to twisted nuclei
• Stromal background is delicately fibrillar
• Scattered mast cells in lesions
Diagnosis
• Localized neurofibroma
• Neuroma
• Fibroma
• Widespread neurofibroma
• MEN 2b/III
• Proteus syndrome
Treatment
• When solitary, excision
• When multiple, verification of the diagnosis, and treatment
directed toward function and/or esthetics
Prognosis
• When isolated, excellent
• When syndrome related, up to 15% risk of malignant transfor-
mation to neurogenic sarcoma
Paget’s Disease
Etiology
• Unknown, although several theories exist including the following:
• Inborn error of connective tissue metabolism
• Paramyxovirus or slow virus infection
• Autoimmune-mediated vascular disorder
• Possible association with alterations involving chromosomes
6 and 18
• Familial form exists
• Nearly one-fifth of cases involve the mandible and maxilla.
• Maxillary and mandibular involvement is usually bilateral and
symmetric.
• Maxilla predominates over mandible by 2:1
• Jaw and skull enlargement common
• Often deep aching pain in affected bone(s)
• Neurologic complications, as follows, in later phases of uncon-
trolled or advanced cases:
• Vertigo, headache
• Auditory/visual disturbances
• Facial paresis
• Monostotic involvement occurs but rarely
• Dental patients often complain of ill-fitting prostheses or slow
separation of teeth.
• Initially ill-defined, often multiple lytic lesions noted
• Later stage shows patchy radiopaque pattern (like cotton
wool)
• Hypercementosis recognizable by “drumstick” appearance of
root outline
• Lamina dura–periodontal membrane space may become
obliterated.
Laboratory Findings
• Increased serum alkaline phosphatase
• Serum calcium and phosphate normal
• Elevated urinary calcium and hydroxyproline levels
• Early phase predominantly has osteoclastic resorption, fibrous
tissue replacement of bone, and prominent blood vessels
• Late phase (sclerotic) has predominantly osteoblastic function,
which results in dense bone with numerous reversal lines
Diagnosis
• Radionuclide imaging to determine extent and distribution of
lesions
• Osteosarcoma
• Acromegaly
Treatment
• Bisphosphonate therapy
• Calcitonin
• Pain control
Prognosis and Complications

• Slowly progressive
• Deformities and neurologic complications in late phases
• Malignant transformation may occur (osteosarcoma) in 1% of
cases, secondary to loss of heterozygosity in chromosome 18q.
Periapical Cemento-osseous Dysplasia

Etiology
• A dysplastic lesion of periodontal membrane origin with no
known cause
• An asymptomatic focus of periapical alteration in the
mandibular region; usually anterior
• May involve apices of one or more teeth
• Noted usually in fourth and fifth decades
• Associated teeth are always vital.
• Initially, findings of a periapical radiolucency include the
following:
• Well-defined, noncorticated border
• Less than 1 cm in diameter
• Lamina dura usually lost
• With aging, radiodensity increases
Diagnosis
• Radiographic features
• Associated tooth (teeth) is vital
• Multiple periapical abcesses
• Florid osseous dysplasia
Treatment
Prognosis
• Excellent
Scleroderma
Etiology
• Autoimmune, with local and systemic or multiorgan effects
secondary to excessive collagen deposition
• Localized sclerosis (morphea) is a distinct disorder sharing only
histologic features with systemic sclerosis.
• Oral findings in progressive systemic sclerosis are as follows:
• Limited oral opening (microstomia)
• Narrowing of lips (“purse string” sign)
• Raynaud’s phenomenon (an early finding)
• Facial skin becomes taut and mask-like.
• Tongue becomes “bound down” and hypomobile.
• Telangiectases over facial skin, lips, tongue
• Prominent antegonial notch on panoramic radiograph
• Variable resorption of condyles and coronoid processes
• Uniform widening of periodontal membrane space
• Root resorption
Diagnosis
• Microscopic features of skin or mucosal biopsy
• Serology: demonstration of anticentromere antibodies or anti-
topoisomerase I (anti-Scl 70)
• Lichen sclerosus
• Submucous fibrosis
• Postradiation scarring
Treatment
• Systemic corticosteroids
• Immunosuppressive agents
• Vasodilators
• Systemic d-penicillamine
• Control of local effects of disease
Prognosis
• Dismal for systemic form
Torus: Palatal and Mandibular

Etiology
• Focal cortical bone overgrowth of unknown cause
• Palatal: slow-growing, asymptomatic, paramedian, nodular
bony mass
• Mandibular: bilateral, smooth and lobular bony masses along
lingual surface in cuspid-premolar area
• Larger lesions may interfere with function or dental prosthesis.
• Larger lesions may show surface ulceration and may lead to
focal osteomyelitis.
• Dense hyperplastic cortical bone with few marrow spaces
Diagnosis
• Clinical presentation is usually diagnostic.
Treatment
• Observation
• Surgical removal under the following conditions:
• If there is interference with seating of prosthesis
• If condition is excessive or symptomatic
Prognosis
• Excellent
Salivary Gland Diseases
Mucocele
Etiology
• Extravasation type
• Physical-traumatic injury to minor gland excretory duct
• Mucus extravasation into periductal soft tissue produces a
local inflammatory response and granulation tissue
“encapsulation.”
• Variant
• Superficial mucocele
• Mucus pool at epithelial–connective tissue junction
• Possibly trauma or systemic (hormonal) etiology
• Lower lip most common site; also buccal mucosa, anterior
ventral tongue
• Painless bluish hue when mucin is near surface
• Often waxes and wanes in size
• Mucus pool surrounded by granulation tissue
• Macrophage and neutrophil response to free mucin
• Focal chronic sialadenitis
Diagnosis
• Presentation
• Hemangioma/varix
• Salivary neoplasm
• Connective tissue neoplasm
Salivary Gland Diseases 207
Treatment
• Excision with associated local minor salivary glands
Prognosis
• Occasional recurrence
Mucus Retention Cyst

Etiology
• Represents dilatation of salivary excretory duct due to
obstruction
• Duct obstruction may be due to a mucous plug or sialolith
formation
• Major or minor salivary glands affected in adulthood
• Asymptomatic, soft mucosal swelling
• Can occur at any intraoral minor salivary gland site, especially
upper lip
• Thin, dilated, epithelial-lined salivary excretory duct
• Lining is cuboidal to columnar with occasional mucus-producing
cells present
• Adjacent salivary gland lobules minimally altered but may
show obstructive inflammatory changes
Diagnosis
• Extravasational mucocele
• Salivary gland neoplasm, especially mucoepidermoid carcino-
ma
Treatment
• Excision of cyst with adjacent gland(s)
Prognosis
• Recurrence is rare.
Necrotizing Sialometaplasia
Etiology
• Local ischemic injury of salivary gland lobules
• May be preceded by trauma or local anesthetic injury, or it
may appear spontaneously
• Both major and minor salivary glands can be affected.
• Hard palate most common site, usually unilateral
• Initially a painful to dysesthetic submucosal swelling
• Ultimately, a central necrotic crater develops.
• May extend to and involve deep soft tissue and palatal bone
• Salivary gland inflammation and lobular necrosis (necrosis is
not always demonstrable on biopsy)
• Ductal squamous metaplasia (bland cytology)
• Lobular architecture of salivary glands persists
Diagnosis
• Salivary gland neoplasm
• Granulomatous disease
Treatment
• Follow-up only
Prognosis
• Excellent
Ranula
Etiology
• Obstruction of the sublingual (usually) or submandibular sali-
vary gland by a sialolith or by trauma
• Secondary to obstruction, extravasation of saliva into the soft
tissue of the floor of the mouth
• Unilateral, fluctuant, soft tissue mass on the floor of the mouth
• Usually has a bluish, slightly translucent quality
• When above the mylohyoid muscle, presentation is intraoral.
• If extravasation extends below the mylohyoid muscle, a plung-
ing ranula forms.
• Occlusal radiographs may demonstrate a suspected sialolith.
Diagnosis
• Demonstration of sialolith
• Soft tissue imaging (T2-weighted magnetic resonance image)
• Aspiration of mucinous salivary fluid
• Excised tissue with granulation tissue lining around mucin pool
• Dermoid cyst
• Salivary gland tumor
• Soft tissue tumor
• Cystic hygroma
• Thymic cyst
Treatment
• Marsupialization as an initial procedure
• Excision of the involved gland (extravasation type)
• Sialolithectomy (in obstructive type)
Prognosis
• No recurrence with sialadenectomy
• Recurrence risk with sialolithectomy secondary to duct scar-
ring or reformation of stone
Sialorrhea (Sialosis)
Etiology
• Varied; may include idiopathic paroxysmal sialorrhea, parkin-
sonism, stomatitis (acute), newly inserted oral appliances,
expectorants, neostigmine, and others
• Excess saliva resulting in drooling
• Angular cheilosis
• Diffuse parotid/submandibular salivary gland enlargement
Diagnosis
• Direct observation and analysis of history
• Flow-rate measurement
Treatment
• Scopolamine
• If related to medication use, an alternate medication should be
chosen, if possible.
Prognosis
• Guarded/indeterminate
Sjögren’s Syndrome
Etiology
• An autoimmune disease resulting in exocrine gland dysfunction
secondary to mononuclear cell infiltration
• Increased prevalence of human leukocyte antigen DR/DQ alleles
• Autoantibody production against nuclear antigens SS-A and SS-B
• No specific agent identified; postulations include the following:
• Potential role for viruses/retroviruses as cofactors
• Possible role of cytokine and hormonal influence on signal
transduction and secretion
• Decrease in exocrine gland function
• Xerostomia
• Xerophthalmia/keratoconjunctivitis sicca
• Salivary and lacrimal gland enlargement (one-third of cases)
• Secondary effects of exocrine dysfunction are as follows:
• Dental caries
• Oral candidiasis
• Ocular/corneal discomfort
• Primary form: exocrine dysfunction dominates
• Secondary form: exocrine dysfunction; other associated
autoimmune conditions—usually rheumatoid arthritis, less
often lupus erythematosus
Diagnosis
• Demonstration of objective xerostomia and xerophthalmia
• Serologic demonstration of associated SS-A or SS-B antibodies
• Correlation of clinical and serologic findings with labial salivary
gland biopsy; demonstration of presence of periductal lympho-
cytic sialadenitis
Differential Diagnosis (Xerostomia/Parotid Gland Swelling)
• Sarcoidosis • Depression
• Human immunodeficiency • Autonomic neuropathy
virus–associated • Graft-versus-host disease
exocrinopathy • Bulimia
• Drug side effects • Alcoholism
• Lymphoma • Diabetes mellitus
Treatment
• Directed at associated connective tissue or autoimmune disease
• Systemic corticosteroids if acute symptoms arise
• Usually symptomatic and preventative therapies are used,
including the following:
• Reduction of oral dryness
• Pilocarpine
• Cemiveline
• Oral moisturizing agents (saliva substitutes)
• Gustatory stimulation
• Ocular moisture replacement
• Saline
• Synthetic glycoprotein solutions
• Carboxymethylcellulose sodium
• Ocular punctual occlusion
• Frequent dental/ophthalmic examinations
Prognosis
• Guarded
• High risk of lymphoma compared with risk in those without
autoimmune disease
Lymphoid Lesions
Burkitt’s Lymphoma
Etiology
• B lymphocyte malignancy associated with genetic mutations:
C-MYC, P53, and others
• Causative association with Epstein-Barr (EB) virus, and malaria
cofactor believed to increase the risk for gene-translocation
accidents
• African form closely associated with EB infection; North
American form less strongly associated
• Rapidly progressive facial asymmetry, chiefly of the mandible
• Proptosis in children may occur in association with maxillary
lesions.
• Pain and paresthesia associated with jaw lesions
• Children predominately affected
• Facial presentation noted in 25% of North American (nonen-
demic) cases; nearly 100% in African children (endemic cases)
• Abdominal (retroperitoneal) presentation usually noted initially
in nonendemic form and in a wider age range than in endemic
form
• Ill-defined radiolucency
• Loss of lamina dura and developmental crypt(s) around
unerupted teeth
• Uniform widening of periodontal membrane space
• Teeth may be displaced, causing malocclusion and/or exfoliation.
Diagnosis
• A diffuse proliferation of small noncleaved lymphoid cells
(B lymphocyte–derived cells)
• Tumor cells have round nuclei and prominent nucleoli.
• Scattered macrophages with abundant pale cytoplasm contain-
Lymphoid Lesions 219
ing pyknotic cellular debris represent the “stars” in the so-

called starry sky appearance.

• Other jaw malignancies of childhood (Ewing’s sarcoma,
osteosarcoma)
• Acute infection
Differential Diagnosis: Microscopic

• Other round cell malignancies of childhood (neuroblastoma,
leukemia, embryonal rhabdomyosarcoma)
Treatment
• Multiagent chemotherapy
• Overall cure rate in children is now up to 90% with intensive,
high-dose fractionated therapy
Prognosis
• Fair
Lymphoepithelial Cyst
Etiology
• Entrapment of oral mucosal epithelium within lymphoid tissue
in foliate papillae, floor of the mouth, ventral tongue, soft palate
• Yellow to white nodule
• Asymptomatic, slow growing
• May drain or decompress spontaneously
• Most common in second then fourth decades
• Overlying mucosa intact, smooth
Diagnosis
• Histologic demonstration of lymphoid tissue with germinal cen-
ters surrounding true cyst lumen filled with epithelial debris
• Lipoma
• Minor salivary gland neoplasm or sialolith
• Mucocele
Treatment
• Excision
Prognosis
• Excellent
Lymphoma
Etiology
• Idiopathic
• Long-term immunosuppression
• ?Post radiation
• Relatively common in head and neck region
• Most common in middle-aged and older individuals
• Mass of reddish blue tissue with ulceration, pain
• Paresthesia of lip when occurring in mandible
• Initial presentation in oral cavity is uncommon (2%)
• Predominant oral sites: palate, gingiva, buccal mucosa, mandible
• May arise within lymph nodes or extranodally in soft tissue
• Ill-defined radiolucency in bone
• Hodgkin’s lymphoma rare in oral cavity
• Burkitt’s lymphoma arises in children.
• Non-Hodgkin’s lymphoma predominates; almost always
B cell type
• Varied, depending upon type/subtype: cell size, pattern, matu-
ration level
• Classification schemes dependent upon microscopic features
• Human immunodeficiency virus–associated lymphomas are
typically diffuse, large cell, high-grade lymphomas.
• EB virus is often evident in tumor cells.
• Revised European American Lymphoma Classification and
Working Formulation are current microscopic classifications
Diagnosis
• Biopsy, immunohistochemical studies
• Flow cytometry
• Staging work-up involves the following:
• Bone marrow biopsy
• Whole body computed tomography scan
• Salivary neoplasm
• Soft tissue tumor (primary)

• Leukemia
Treatment
• Dependent upon extent/clinical stage and microscopic features
• For localized (stage I) disease: radiation therapy
• Chemotherapy or combined chemotherapy-radiation therapy
for more widespread disease
• Very-low-grade lesions may be observed only since treatment
typically has no effect on outcome.
Prognosis
• Dependent upon clinical stage and histologic subtype; acquired
immunodeficiency syndrome–associated lymphoma has a poor
outcome
• Very-low-grade lesions have excellent prognosis
Myeloma
Etiology
• Neoplastic proliferation of malignant plasma cells
• Monoclonal immunoglobulin (κ or λ light chain) production
• Occurs exclusively in adulthood (males 2:1 over females)
• Bone pain and paresthesia
• Mucosal involvement may occur as polypoid to lobular masses.
• Purpura and woody induration of the tongue (macroglossia)or
gingiva may be the initial manifestation.
• Solitary presentation invariably becomes multiple myeloma.
• The extramedullary form occasionally becomes multiple
myeloma.
• Sharply defined radiolucencies, usually of many bones
• Absence of marginal hyperostosis or opaque lining
Laboratory Findings
• Monoclonal gammopathy by serum electrophoresis
• Bence Jones proteinuria
• Plasma cells in bone marrow aspirate
• Monotonous, diffuse plasma cell proliferation
• Variable levels of differentiation and mitotic activity
• Immunohistochemical demonstration of monoclonality (κ or λ
light chains)
Diagnosis
• Biopsy
• Immunohistochemical evaluation
• Lymphoma
• Primary osseous tumor
• Traumatic bone cyst
Treatment
• Chemotherapy
• Local radiation therapy
Prognosis
• Poor
Cysts
Aneurysmal Bone Cyst

Etiology
• Unknown
• Possibly represents a vascular response/repair to jaw injury
(an arteriovenous malformation)
• Three phases: incipient, destructive, stabilization
• Bony expansion and occasionally mild pain
• Chiefly occurs in mandible
• Female predilection
• Expansile, multiloculated, destructive bony lesion
• Surrounding bone may be sclerotic
• Angiogram demonstrates intense vascularity
Diagnosis
• Radiographic lytic lesion
• Honeycombed quality of large vascular sinusoidal spaces
and bony septa
• May be confused microscopically with central giant cell
granuloma
• Ameloblastoma
• Hemangioma
• Giant cell granuloma
Treatment
• Excision to en bloc resection
Prognosis
• Good to excellent
Cysts 227
Calcifying Odontogenic Cyst

Etiology
• An odontogenic cyst with characteristic microscopic pattern
• May be noted in association with other odontogenic tumors
• Origin is residual odontogenic epithelium in the jaws; stimulus
unknown
• Usually a unilocular, well-defined radiolucency, chiefly of maxilla
• Scattered opacities seen in up to 50% of cases
• May be associated with the crown of an unerupted tooth
• An extraosseous form may occur (usually anterior to first molar)
• May be more solid than cystic (odontogenic ghost cell tumor)
• Well-defined radiolucency or lucency with opaque foci (dys-
trophic calcification of keratin produced by lining epithelium)
• Tooth displacement or root resorption may be seen.
Diagnosis
• Stratified squamous lining with prominent basal layer
• Budding or extension of epithelium into the cyst wall may be
noted.
• Characteristic ghost cell keratinization required for diagnosis
• Ghost cells may undergo dystrophic calcification.
• Foreign body reaction may occur when ghost cells come in
contact with connective tissue.
• The solid or tumorous form shares microscopic features with
ameloblastoma.
Differential Diagnosis: Radiographic

• Calcifying epithelial odontogenic tumor
• Ameloblastic fibro-odontoma
Cysts 229
Treatment
• Enucleation/excision
• If noted in association with another odontogenic tumor, con-
sideration must be given to the behavior of the accompanying
lesion.
Prognosis
• Some recurrence potential, especially in association with solid
lesions
• Overall prognosis is very good
• Excellent prognosis for peripheral (gingival) lesions
Dental Lamina Cyst (Bohn’s Nodules;

Gingival Cyst of Newborn)
Etiology
• Cystic degeneration of residual dental lamina/odontogenic
epithelium
• Found in over 80% of newborns
• Small (1–2 mm), usually multiple, yellow-white nodules over
the alveolar crest in neonates
• Usually involute following spontaneous cyst rupture
Diagnosis
• Appearance and location
• Histologically, parakeratinized epithelial lining with keratin-
filled cyst cavity is noted.
• Eruption cyst
Treatment
Prognosis
• Excellent
Cysts 231
Dentigerous Cyst
Etiology
• A developmental odontogenic cyst arising subsequent to sepa-
ration between dental follicle and the crown of an associated
unerupted tooth
• Proliferation of reduced enamel epithelium lining the follicle,
with fluid accumulation between epithelium and impacted
tooth crown
• Alternatively, degeneration of the stellate reticulum component
of the enamel organ occurs during odontogenesis.
• Most commonly involves frequently impacted teeth: mandibu-
lar third molars, followed by maxillary canines
• Usually noted during second and third decades
• Asymptomatic; discovered on routine radiographic examination
• Painless jaw/alveolar expansion may occur; cortex is thinned
and rarely perforated
• Well-defined radiolucency enclosing crown of unerupted tooth
• Corticated/opaque margins unless infected
• May produce root resorption of adjacent erupted teeth
• Usually unilocular; less commonly multilocular
Diagnosis: Microscopic
• Cysts without secondary inflammation
• Thin, cuboidal, nonkeratinized epithelial lining two cell
layers thick with flat epithelial–connective tissue interface
• Loosely arranged collagen bundles, occasionally containing
scattered odontogenic epithelial rests
• Cysts with secondary inflammation
• Hyperplastic, nonkeratinized squamous epithelial lining
with epithelial ridge development
• Variable chronic inflammatory cell infiltrate within
condensed collagen stroma
Cysts 233

• Ameloblastoma
Treatment
• Cyst enucleation and extraction of associated tooth
• Marsupialization prior to excision may be considered if the
cyst is very large.
Prognosis
• Excellent
• Possible complications
• Pathologic fracture with large lesions
• Neoplastic transformation of epithelial lining
(ameloblastoma and, rarely, squamous cell carcinoma)
Dermoid Cyst
Etiology
• Cystic degeneration of entrapped epithelium within the midline
fusion zone between the first and second branchial arches
• Alternative etiology relates to in utero traumatic epithelial
implantation into floor of mouth area
• Slowly enlarging, usually asymptomatic, sublingual or floor-of-
mouth mass
• May present as a soft and compressible paramedian swelling or
deformity
• Overlying mucosa/skin is thinned, but is otherwise unremarkable
• May have doughy consistency because of sebum and/or keratin
in cystic cavity
• Epithelial lining (stratified squamous)
• Cyst contents may include keratin debris, hair follicles/hair,
and sebaceous and sweat glands.
• Rarely find gastric mucosal characteristics present in cyst lining
Diagnosis
• Aspiration may yield cellular debris, sebum, keratin, mucus
• Histologic demonstration of hair follicles, sebaceous glands,
keratinizing cystic lining
• Cellulitis of odontogenic origin
• Sublingual sialadenitis
• Ranula (superficial or deep/plunging)
Treatment
• Intraoral surgical excision
Prognosis
• Excellent
Cysts 235
Eruption Cyst
Etiology
• Soft tissue cyst of attached gingiva secondary to fluid accumu-
lation within the follicular space of an unerupted tooth
• Gingival swelling on the alveolar crest
• Usually soft, translucent to bluish (“eruption hematoma”)
Diagnosis
• Location
• Radiographic demonstration of erupting tooth
• Gingival cyst
Treatment
• Usually none is necessary as tooth typically erupts through
lesion
• Possibly unroof cyst to facilitate eruption
Prognosis
• Excellent
Cysts 237
Glandular Odontogenic Cyst

Etiology
• A developmental odontogenic cyst
• A unique jaw cyst with microscopic evidence of glandular dif-
ferentiation
• Slow growing; may be expansile
• Located chiefly in the anterior mandible
• May present a lateral periodontal relationship
• Usually a multilocular cystic radiolucency
• Sharply defined with hyperostotic margins
• May be extensive, locally invasive; may perforate cortical bone
Diagnosis
• Radiographic qualities
• Incisional biopsy results show cystic epithelium with mucous
cells and pseudoduct formation
• Giant cell lesion
• Ameloblastoma
• Lateral periodontal cyst
Treatment
• Excision, peripheral ostectomy
• En bloc excision
• Primary reconstruction
Prognosis
• Recurrence may be associated with conservative management.
Cysts 239
Lateral Periodontal Cyst

Etiology
• Stimulus unknown
• Dental lamina remnant proliferation within the alveolar
segment of the jaw, separate from the periodontal ligament
• Asymptomatic
• Usually occurs in fourth decade and beyond
• Usually in mandibular canine/premolar region (65%)
• In the maxilla, the lateral incisor area predominates.
• Well delineated, round to ovoid lucency with thin, opaque
(corticated) margin
• Located lateral to vital tooth roots
• Usually unilocular; may be multilocular (botryoid odontogenic
cyst)
Diagnosis
• Thin, nonkeratinized epithelial lining
• Nodular epithelial thickening along cyst lining
• Lining cells are cuboidal with interspersed clear glycogen-filled
cells.
• Inflammatory, lateral radicular cyst
• Primordial cyst/odontogenic keratocyst
• Odontogenic tumor
• Glandular odontogenic cyst
Treatment
• Conservative enucleation
• The botryoid variant requires more aggressive curettage.
Prognosis
• Recurrence uncommon
• Increased risk of recurrence with botryoid variant; longer-term
follow-up necessary
Cysts 241
Nasopalatine Duct Cyst

Etiology
• Developmental, nonodontogenic cyst
• Cystic degeneration of epithelial remnants of the vestigial
nasopalatine duct
• Usually develops in adulthood
• May be incidental on routine dental radiographs
• Palatal mass with tenderness and drainage
• Adjacent teeth are vital.
• Well-defined, median-paramedian radiolucency in anterior
maxillary midline, greater than 5 to 6 mm in diameter
• Border usually sclerotic
• Round, ovoid, or heart shaped
Diagnosis
• Biopsy confirmation
• Apical/radicular cyst
• Other odontogenic cysts
Treatment
• Enucleation
Prognosis
• Rarely recurs
Cysts 243
Nevoid Basal Cell Carcinoma Syndrome

Etiology
• Autosomal-dominant condition
• Loss of heterozygosity at chromosome 9q22.3
• Mutation of PTCH tumor suppressor gene
• Multiple jaw cysts (odontogenic keratocysts)
• Numerous cutaneous basal cell carcinomas, which arise early
in life and are independent of sun exposure
• Bifid ribs
• Calcification of falx cerebri
• Ocular hypertelorism
• Mandibular prognathism
• Broad nasal bridge
• Medulloblastoma
• Palmar and plantar pits
• Multiple jaw radiolucencies
• Lamellar calcification of falx cerebri
• Bifid rib on abdominal radiograph
Diagnosis
• Radiographic and clinical findings
• Other syndromes, such as the following:
• Charcot-Marie syndrome
• Waardenburg’s syndrome
Treatment
• Excision of basal cell carcinomas and odontogenic keratocysts
• Excision of other related aggressive tumors at other sites
Prognosis
• Guarded
Cysts 245
Odontogenic Keratocyst
Etiology
• A benign, aggressive developmental odontogenic cyst; may be
associated with mutation of PTCH tumor suppressor gene
• 5 to 15% of odontogenic cysts
• Usually occurs sporadically as an isolated finding
• Approximately 5% are associated with nevoid basal cell
carcinoma.
• 5% of patients have multiple odontogenic keratocysts (OKCs)
and no syndrome
• Can occur in any area of maxilla or mandible
• Rarely may arise in gingival soft tissue only (peripheral)
• Mandible is preferred site in 65 to 78% of cases
• Often (40%) seen in a dentigerous relationship
• Discrete radiolucency, usually in relation to teeth (apical, lateral
radicular, pericoronal to impacted tooth)
• May be unilocular to multilocular
• Thin, parakeratinized epithelial lining (6–10 cells thick)
• Wavy, corrugated surface configuration
• Prominent, palisaded, cuboidal to low-columnar basal cell layer
• Basal layer “budding” into fibrous stroma is seen occasionally
• Satellite or daughter cyst formation noted frequently
Diagnosis
• Odontogenic cysts: dentigerous, radicular, lateral periodontal,
or glandular odontogenic
• Nonodontogenic cyst: nasopalatine duct
• Odontogenic tumors: ameloblastoma, myxoma
Cysts 247
• Giant cell granuloma

• Central mucoepidermoid carcinoma
Treatment
• Excision with curettage of bony confines
Prognosis
• The recurrence rate varies from 10 to 30% (solitary OKCs).
• Recurrence rates are greatest in patients with a syndrome.
Primordial Cyst
Etiology
• A developmental odontogenic cyst arising from cystic degener-
ation of the enamel organ prior to formation of hard tissue
• Invariably has the microscopic appearance of odontogenic
keratocyst
• Rare
• Radiolucent lesion of jaw
• Occurs in place of a tooth, usually a third molar
• A well-defined radiolucency
• Most commonly in the posterior mandibular quadrants
Diagnosis
• Radiograph shows a cyst instead of a tooth
• Histologically an odontogenic keratocyst
• Other odontogenic cyst
• Central giant cell granuloma
Treatment
• Enucleation with bone curettage
Prognosis
• Significant recurrence rate
• Long-term follow-up mandated
Cysts 249
Radicular Cyst
Etiology
• Preceded by periapical granuloma; arises as follows:
• Secondary to necrosis of dental pulpal tissue
• Stimulation of epithelial network (Malassez’s rest) at tooth
root apex results in cystification
• Cyst growth continues secondary to effects of osmotic gradient
across epithelial lining layers, mediators of inflammation, and
epithelial proliferation
• Asymptomatic unless there is an acute exacerbation
• Usually a limited process at root apex or lateral to root surface
• Radiograph shows a round and well-defined lucency, usually
with a sclerotic margin.
• Generally 1 cm or less across, but can be significant in size
• Root resorption uncommon
• Stratified squamous epithelial lining
• Lumen filled with cell debris, fluid, cholesterol
• Connective tissue wall with mixed inflammatory infiltrate
Diagnosis
• Documentation of nonvital tooth
• Radiograph shows alteration of apical bone
• Periapical granuloma
• Odontogenic and nonodontogenic tumors
Treatment
• Endodontic therapy or
• Periapical surgery and biopsy or
• Tooth extraction and biopsy
Cysts 251
Prognosis
• Excellent
• Occasional recurrences
Thyroglossal Duct Cyst

Etiology
• Cystic change associated with thyroglossal duct remnants that
failed to involute (tenth week of development)
• Rarely may be hereditary in origin (autosomal dominant or
recessive)
• Soft, painless, and slowly enlarging mass in anterior midline of
the neck of children and young adults
• Usually unilocular as seen by ultrasound examination
• Mass is usually mobile
• Most occur above the hyoid bone.
• May involve the tongue
• Cyst lining of squamous, transitional, ciliated columnar epithe-
lium composite
• The cyst wall may contain residual thyroid tissue.
Diagnosis
• Ultrasonography
• Clinical presentation of midline neck mass
• Histopathology
• Base-of-tongue carcinoma
• Base-of-tongue salivary tumor
• Thyroid carcinoma arising within cyst
Treatment
Prognosis
• Excellent
• Recurrence owing to tortuous morphology
• Rarely, carcinomatous transformation of duct lining or rem-
nants of thyroid parenchyma are noted.
Cysts 253
Traumatic Bone Cyst

Etiology
• Unknown in most cases
• May be due to traumatic injury producing intramedullary
hemorrhage and subsequent clot resorption
• Alternative theory suggests degeneration of primary intrabony
pathology
• Peaks in second decade
• Usually in body of mandible
• Painless in most cases
• Swelling noted in one-fourth of cases
• Clearly defined radiolucency
• Margins may be uneven but clear.
• May extend between tooth roots creating a scalloped pattern
Diagnosis
• Clinical finding of an empty bony space (pseudocyst)
• Collagen and fibrin line the dead space (no epithelium).
• Lamellar bone may be noted along the bony margin.
• Fibro-osseous lesion (early)
• Hemangioma
Treatment
• Surgical exploration
• Observation for resolution
Prognosis
• Excellent
• Small risk of recurrence
Cysts 255
Odontogenic Tumors
Adenomatoid Odontogenic Tumor

Etiology
• Derivation from epithelial component of the enamel organ
• Represents less than 10% of odontogenic tumors
• Biologic behavior allows for distinction from ameloblastoma
• Narrow age range, 5 to 30 years, with most cases noted during
second decade
• Female predilection
• Anterior jaw location common
• Association with unerupted tooth
• Asymptomatic; occasionally produces expansion of alveolar
bone
• Rarely occurs in gingival soft tissue (peripheral)
• May produce root divergence of adjacent teeth
• Well defined, unilocular, often adjacent to crown of unerupted
tooth
• Opaque foci may be scattered within the lucency in a
“snowflake” or “salt and pepper” pattern.
• Characteristic intraluminal/intracystic growth with well-
defined capsule
• Dual cell population: spindle cells and cuboidal to columnar
cells forming tubules or pseudoducts
• Foci of dystrophic calcification or eosinophilic droplets may
be noted.
Diagnosis
Odontogenic Tumors 257
• Dentigerous cyst
• Calcifying odontogenic cyst
• Lateral root cyst
• Calcifying epithelial odontogenic tumor
Treatment
• Enucleation
Prognosis
• No recurrence
Ameloblastic Fibroma and

Ameloblastic Fibro-odontoma
Etiology
• Ameloblastic fibroma: a benign mixed odontogenic tumor with
concomitant epithelial and mesenchymal neoplastic proliferation
• Ameloblastic fibro-odontoma: as for ameloblastic fibroma with
the addition of an odontoma
• Spontaneous; no known cause for either
• Noted mostly in first and second decades
• Approximately 70% in mandible, usually posterior region
• No gender predilection
• May cause jaw expansion
• Asymptomatic
• Well defined with hyperostotic margin
• Unilocular to multilocular
• Often associated with an unerupted tooth
• Ameloblastic fibro-odontoma has opaque component(s) related
to enamel and dentin in the odontoma component
Diagnosis
• Lobulated, cellular mesenchymal component with proliferating
odontogenic epithelium in cords and islands
• Enamel matrix, dentin formation associated with odontoma
(when present)
• Ameloblastoma
Treatment
• Conservative surgical excision/curettage
Prognosis
• Excellent
Ameloblastoma
Etiology
• A benign, aggressive jaw tumor of odontogenic epithelial (ecto-
dermal) origin; the most common odontogenic tumor after the
odontoma
• Incidence of 0.3 cases per million people
• Peak incidence during third to fifth decades
• 80% occur in the mandible, chiefly in molar and ramus region
• Often presents in association with unerupted third molar teeth
• May produce marked deformity, facial asymmetry
• Extraosseous or peripheral variant arises in gingival tissues of
older adults (fifth to seventh decades)
• Typically slow growing, but persistent
• Osteolytic or radiolucent with sclerotic, smooth, even borders
• May be unilocular to multilocular
• Root resorption or tooth displacement may be seen.
• Can expand affected jaw in any plane
• Cortical perforation may occur.
Diagnosis
• Sheets, strands, islands of odontogenic epithelium
• Peripheral layer of cuboidal to columnar ameloblast-like cells
enclosing a cell population analogous to stellate reticulum of
the enamel organ
• Cystic degeneration common within stellate reticulum component
• Several histologic patterns described have no clinical relevance.
• A biologic variant, cystic (unicystic) ameloblastoma, occurs in
younger patients; has a less aggressive clinical course and is
managed more conservatively
• Malignant variants rarely seen
Treatment
• Varies with subtype, size, location
• Solid/multicystic lesions generally require local excision or
resection.
• The cystic variant requires local excision, as recurrences may
follow curettage only
Prognosis
• Generally good; recurrence rates higher with conservative
treatment
• Recurrence rates of up to 15% following marginal resection
• Very good prognosis for cystic ameloblastoma
• Long-term follow-up necessary
Calcifying Epithelial Odontogenic Tumor

Etiology
• A benign odontogenic tumor of uncertain histogenesis
• Stratum intermedium component of enamel organ is favored
cell of origin
• Chiefly in posterior mandible
• Painless, slow growing
• Mean age of occurrence is approximately 40 years
• Occasional soft tissue origin (peripheral) noted as a sessile
gingival mass
• Jaw expansion a common clinical presentation
• Usually noted in association with an impacted tooth
• Multilocular; most often with mixed radiolucent and
radiopaque features
• Impacted tooth often obscured by tumor-associated calcification
• Margins may be well defined or sclerotic and vague.
Diagnosis
• Biopsy findings of polyhedral epithelial cells, nuclear pleomor-
phism, amyloid material, and concentric calcifications with
epithelial islands
• When radiolucency predominates: dentigerous cyst, odonto-
genic keratocyst, ameloblastoma, odontogenic myxoma
• With mixed radiolucent and radiopaque features: calcifying
odontogenic cyst, adenomatoid odontogenic tumor, ameloblas-
tic fibro-odontoma, fibro-osseous lesion, osteoblastoma
Treatment
• Local, conservative excision including a thin rim of normal
bone (so-called ostectomy) versus conservative en bloc removal
• Peripheral lesions with a narrow periphery of normal-appearing
mucosa
Prognosis
• Very good
• Recurrence rate is low, from 10 to 15%
• Long-term follow-up recommended
Odontogenic Myxoma
Etiology
• A benign odontogenic tumor
• Unknown origin
• A lesion of adulthood (average occurrence at 30 years)
• Equal male:female and mandible:maxilla occurrences
• Wide age range: second through sixth decades
• May produce jaw expansion
• Well-defined, unilocular to multilocular radiolucency
• Loculi range from small “honeycomb” to large “soap bubble”
shapes
• Cortical thinning may be present with larger lesions.
• Perforation of the cortex is uncommon.
• Minimal cellularity, myxoid background
• Variable amounts of collagen
• Scattered residual bony trabeculae
• Odontogenic epithelial rests are rarely noted.
Diagnosis
• Other odontogenic tumor: ameloblastoma
• Odontogenic cysts: odontogenic keratocyst, dentigerous cyst,
glandular odontogenic cyst
Treatment
• Excision with bony curettage
• Large lesions may require en bloc resection.
Prognosis
• Good
• Can be aggressive rarely
• Recurrences not uncommon, secondary to gelatinous quality
and lack of capsule
Odontoma
Etiology
• A hamartomatous or benign mixed odontogenic tumor of the jaw
• Composed of enamel, dentin, cementum, and pulp tissue
• Two forms, as follows:
• Complex: a randomly arrayed mixture of dental tissues with
no gross resemblance to a tooth
• Compound: multiple, tooth-like structures
• Mean age of occurrence, 12 to 16 years
• Asymptomatic, usually small and discovered incidentally
• Jaw expansion may be present with large lesions.
• Presence may be heralded by an over-retained primary tooth or
by alveolar swelling.
• Well-localized, mixed radiolucent and radiopaque lesion
• Within alveolar segment of jaws
• Complex form most commonly noted in mandibular molar area
• Compound form favors anterior jaw region, usually the maxil-
la; may contain a few small teeth or large numbers of tiny
tooth-like structures
Diagnosis
• Radiographic presentation
• Histologic demonstration of dental hard tissues
• Ameloblastic fibro-odontoma
• Adenomatoid odontogenic tumor
• Calcifying odontogenic cyst
• Focal sclerosing osteitis, osteoma
Treatment
• Conservative excision/curettage
Prognosis
• Excellent
Peripheral Odontogenic Fibroma

Etiology
• A benign proliferation neoplasm of fibroblastic and odonto-
genic epithelial origin
• Asymptomatic, firm, slow-growing mass of the attached gingiva
• Overlying mucosa unremarkable and intact
• Sessile growth pattern
• Usually along facial or buccal aspect of gingiva
• Calcifications may be present radiographically.
• Underlying alveolar bone is spared.
• Uncommon to rare
• Also seen centrally (within bone)
Diagnosis
• Fibrous to myxoid stromal tissue
• Scattered islands and strands of odontogenic epithelium
• Some cells may be vacuolated.
• The degree of epithelial proliferation may vary from minimal
to prominent.
• Peripheral fibroma
• Peripheral ameloblastoma
Treatment
• Excision: local and conservative
Prognosis
• Excellent
Benign Nonodontogenic Tumors
Carotid Body Tumor

Etiology
• Rare neoplasm arising from nonchromaffin paraganglia in
carotid artery bifurcation
• Heredofamilial (autosomal-dominant) form can occur (in less
than 10%)
• Can be multiple, bilateral, or multicentric
• Typically presents as a mass in the lateral neck
• May be associated with bruit, hoarseness, dysphagia
Diagnosis
• Ultrasonography as a screening measure
• Angiography of both carotid systems
• Vagal nerve sheath tumor
Treatment
• Surgical removal
• Radiation therapy
• Combined surgical and radiotherapy
Prognosis
• Generally good
• Can be locally invasive
• May metastasize in 5 to 25% of cases
Benign Nonodontogenic Tumors 271
Exostosis
Etiology
• Unknown
• Probable reactive phenomenon (stimulus undetermined)
• Asymptomatic, bony, nodular masses
• Cortical bone enlargement of the jaws; usually bilateral and
symmetric
• Usually multiple; slow growing
• Most commonly along buccal/facial aspects of the maxillary
and mandibular alveolar ridge
• Overlying mucosa intact, unremarkable
• Usually develops in adults
Diagnosis
• May appear radiographically as homogeneous opacities
• Periostitis
• Periosteal/parosteal osteosarcoma
Treatment
• None required
• May need to be removed for prosthesis (denture) construction
Prognosis
• Excellent
Juvenile Ossifying Fibroma

Etiology
• A rapidly evolving variant of ossifying fibroma of the young
• Cause unknown
• Onset between 5 and 15 years of age
• Rapid growth over several weeks
• Maxilla and paranasal areas predominate
• Tooth displacement common
• Well-defined radiolucency
• Focal mineralization may be noted.
• Adjacent bone may be eroded or destroyed.
• Prominent stromal cellularity
• Woven bone and/or psammomatous calcifications
• Plump osteoblast rimming
Diagnosis
• Correlation of histologic and radiographic findings
• Osteosarcoma
Treatment
• Wide local excision or resection
• Reconstruction
Prognosis
• Recurrence rate of 30 to 50%
Langerhans Cell Disease

(“Histiocytosis X,” Idiopathic Histiocytosis)
Etiology
• Unknown
• Proliferation of Langerhans’ cells (immune surveillance cells)
normally found in skin, mucosa, bone marrow, and lymph nodes
• A broad spectrum, typically divided into three subsets, as follows:
• Unifocal or multifocal chronic disease of bone (eosinophilic
granuloma)
• Widely disseminated chronic disease of bone and soft tissue
(Hand-Schüller-Christian disease)
• Acute, disseminated disease with bone marrow involvement
(Letterer-Siwe disease)
• Most arise in childhood; eosinophilic granuloma often arises in
adolescents and adults.
• Jaw lesions noted in up to 20% of cases with tenderness, loose
teeth (focal to segmental), gingival inflammation, and friability
• Bone lesions often punched out, sharply circumscribed
• “Floating teeth” appearance with alveolar bone involvement
• Skeletal survey should be performed to rule out multiple bone
involvement
Diagnosis
• Radiographic demonstration of lytic bony lesions
• Infiltrate of mononuclear cells, often with clefted nuclei
• Often accompanied by a variety of other cell types, including
eosinophils, lymphocytes, giant cells, plasma cells
• Immunohistochemical demonstration of CD1a staining
• Langerhans’ cells also stain for S-100 protein, although the
antibody is less specific.
• Ultrastructural demonstration of cytoplasmic racquet-shaped
Birbeck granules
• Cat-scratch disease
• Juvenile xanthogranuloma
Differential Diagnosis: Radiologic

• Juvenile periodontitis, endocrinopathies, hypophosphatasia,
leukemia, bony malignancy (primary/metastatic)
• In adults: myeloma
Treatment
• Localized variant
• Surgical curettage of bony lesions
• Low-dose radiation therapy of inaccessible lesions
• Widespread variants
• Chemotherapy including methotrexate, vincristine,
cyclophosphamide
• Bone marrow transplantation for resistant/recurrent disease
Prognosis
• Varies with form of disease, as follows:
• Localized variant: very good
• Disseminated variant: fair to poor
Ossifying Fibroma
Etiology
• A benign fibro-osseous lesion of bone
• Cause unknown
• Expansile lesion of bone
• Cortices intact
• May produce deformity, malocclusion, dysfunction
• Mandibular lesions are more common than are maxillary.
• Well-delineated, smooth contours
• Quality varies from lucent to opaque
• Margins may be sclerotic.
• Can resorb roots and displace teeth
• May displace mandibular canal
• Fibrovascular stroma
• Islands/trabeculae of osteoid, woven bone
• Cementum droplets may be present.
Diagnosis
• Correlation of histologic and radiographic findings

• Odontogenic cyst
• Giant cell lesion
Differential Diagnosis: Histologic

• Fibrous dysplasia (must have clinical-pathologic correlation)
Treatment
• Conservative excision
• Enucleation with peripheral bony curettage
Prognosis
• Excellent
Osteoma
Etiology
• Sporadic form is idiopathic
• May be a component of Gardner’s syndrome
• Excludes maxillary and mandibular tori
• Sporadic form with frontal and sphenoid sites predisposed
• May be multiple
• Solitary lesions rare in jaws
• Well circumscribed, dense, sclerotic
• May be subperiosteal or medullary
Diagnosis
• Microscopic features: normal cortical and trabecular bone
• Tori, exostoses
• Osteoblastoma
• Focal sclerosing osteitis
Treatment
• Usually none
• Local resection, if compromising
Prognosis
• Excellent
• Little recurrence potential
• When associated with Gardner’s syndrome, malignant conver-
sion of intestinal polyps is assured.
Peripheral Ossifying Fibroma

Etiology
• A reactive hyperplasia of the gingiva; may be related to chronic
irritation
• Periodontal ligament/membrane origin postulated
• Exclusive gingival location; commonly interdental
• Nodular, sessile to pedunculated, usually ulcerated mass
• Slow growing; may rarely displace teeth
• Usually in young adults and adolescents
• Early lesions may bleed easily.
• Anterior maxillary arch is favored site
Diagnosis
• Central islands or trabeculae of bone/cementum
• Fibroblastic proliferation in a sheet-like configuration
• Usually ulcerated with granulation tissue base
• Peripheral odontogenic tumor
• Osteosarcoma/chondrosarcoma
• Metastatic neoplasm
Treatment
• Excision including underlying periosteum or associated peri-
odontal ligament
Prognosis
• Recurrence occasionally seen; believed to be related to incom-
plete excision
Inflammatory Diseases
Angioedema
Etiology
• Usually triggered by ingested antigens (eg, shellfish, nuts,
fruits, medications)
• Mechanism associated with immunoglobulin E (IgE)-mediated
mast cell degranulation with subsequent histamine release
• Drug reactions resulting in release of inflammatory mediators
(bradykinin)
• Some cases have a genetic basis: C1 esterase inhibitor deficien-
cy or inhibitor dysfunction (autosomal recessive)
• May be correlated with disease states characterized by the
presence of circulating immune complexes
• Soft, diffuse, painless swelling of face, lips, and neck
• Overlying skin and oral mucosa appear noninflamed
• Mucosa may become secondarily erythematous, ulcerative, or,
rarely, vesicular
• Usually short-lived (24– 48 hours)
Diagnosis
• Nonspecific histology
• Correlation of history and clinical findings
• Trauma (physical, cold)
• Cellulitis
• Acute contact stomatitis
• Melkersson-Rosenthal syndrome (early stages)
• Orofacial granulomatosis (early stages)
Treatment
• Elimination of possible etiologic/precipitating factor(s)
• Antihistamines, corticosteroids, adrenaline
Inflammatory Diseases 285
Prognosis
• Good to excellent
Cheilitis Granulomatosa
Etiology
• Isolated, idiopathic, and chronic lip enlargement
• May be an incompletely expressed or oligosymptomatic form
of Melkersson-Rosenthal syndrome
• One or both lips may be diffusely enlarged and nontender.
• Episodic swelling initially, with progression to a persistent
enlargement
• Less often, superficial labial exfoliation or surface
weeping/crusting may be noted.
• Lip swelling may herald similar changes of the gingiva, buccal
mucosa, or palate.
• May be associated with Crohn’s disease, sarcoidosis, contact
sensitivity, dental abscesses
• Demonstrates noncaseating epithelioid granulomas
• Absence of organisms
Diagnosis
• History of intermittent to persistent asymptomatic lip swelling
• Lip or soft tissue biopsy (involved gingiva)
• Rule out sarcoidosis (chest radiograph, serum angiotensin-
converting enzyme levels)
• Patch testing for contact allergens
• Dental radiographs to rule out asymptomatic periapical
pathology
• Angioedema
• Cellulitis/erysipeloid reaction
• Sarcoidosis
• Melkersson-Rosenthal syndrome
• Cheilitis glandularis
Treatment
• Local intralesional triamcinolone injections under local anes-
thesia
• 5 to 10 mg total dose in depot fashion every 3 to 4 weeks to
achieve response
• Local treatment may be coupled with an initial systemic course
of glucocorticoids.
• Clofazimine 100 mg daily for 60 days with reduction to a
maintenance dose of 30 mg on alternate days
• Metronidazole may also be effective at 250 mg three times
daily for 1 month. This may be coupled with intralesional cor-
ticosteroid placement.
• Dapsone may be effective (as per dermatitis herpetiformis dos-
ing)
• Surgical reduction (cheiloplasty) may be necessary.
Prognosis
• Guarded
• Must remain aware of possible neurologic manifestations, oph-
thalmologic involvement, psychological effects
Drug-Induced Stomatitis
(Stomatitis Medicamentosa)
Etiology
• Oral changes found in approximately 5% of those with cuta-
neous reaction to drugs
• Mucosal alterations may result from the following:
• Myelosuppression
• Direct cytotoxic or cytostatic effect(s) on dividing epithelial
cells
• Xerostomic effects
• Alterations of oral microbial flora
• Painful, erythematous, erosive, or ulcerative lesions
• Nonkeratinized locations often affected initially
• Fixed form of drug-associated eruptions relatively uncommon
intraorally
• Pseudomembranous necrotic surface may be noted
Diagnosis
• History
• Chemical or thermal burn
• Candidiasis
Treatment
• Identification and withdrawal of offending drug
• Symptomatic management including topical preparations
(see “Therapeutics” section)
• Systemic corticosteroids if mucosal reaction is not related to

antineoplastic treatment
Prognosis
• Generally excellent
Garré’s Osteomyelitis
Etiology
• Chronic, low-grade, dentoalveolar infection
• Resultant bony inflammation extends to the periosteum, pro-
ducing a reduplication of the cortex (“onion skin” effect).
• Usually an asymptomatic, unilateral, mandibular, bony hard
asymmetry
• Limited to children and young adults
• Medullary mottling with (lucent and opaque) ill-defined margins
• Periosteal-cortical expansion
• Occlusal radiograph shows concentric or parallel layering of
cortex
Diagnosis
• Carious mandibular tooth, usually first permanent molar
• Biopsy results showing periosteal osteoblastic reaction, mini-
mally inflamed fibrous marrow
• Ewing’s sarcoma
• Langerhans cell disease (histiocytosis X)
• Osteosarcoma
• Fibro-osseous lesion
• Metastatic disease
Treatment
• Elimination of the infected focus (carious tooth to be extracted
or filled)
• Antibiotic administration early in treatment phase
Prognosis
• Good
Gingivitis
Etiology
• Variable
• Most are microbiologic or plaque associated (simple marginal
gingivitis).
• Some are modified by hormonal changes, such as those in
pregnancy (pregnancy gingivitis).
• Fusospirochetal gingivitis plus poor oral hygiene and poor nutri-
tion are associated with acute necrotizing ulcerative gingivitis.
• Rarely, some forms are associated with contact allergy (“plasma
cell gingivitis”).
• Dependent on etiology, as follows:
• Plaque associated: marginal inflammation to more
generalized erythema and blunting of interdental papillae
with rolled margins
• Hormonally related: diffuse erythema and hyperplasia
• Fusospirochetal: necrotic, blunted, ulcerated interdental
papillae with spontaneous bleeding; foul odor
• Allergy based: hyperplastic and bright red, granular to
velvety surface alteration
Diagnosis
• Identification of cause
• Patch testing for contact allergens
• Acquired immunodeficiency syndrome–associated periodontal
disease
• Oral lichen planus
Treatment
• Local débridement and chlorhexidine rinses in cases of bacterial
origin
• Reduction of hormonal dosage
• Elimination of allergen
Prognosis
• Excellent
Median Rhomboid Glossitis

Etiology
• A benign, inflammatory condition
• Often related to yeast colonization (erythematous candidiasis)
• Inflammatory process noted in response to overlying Candida
population
• Exact mechanism is unclear
• Well-defined, asymptomatic erythematous patch on dorsum of
tongue
• Paramedian erythema, usually with focal atrophy of filiform
papillae
• Chronic forms may become multinodular.
• Rarely may be hyperkeratotic
• May be mistaken for a benign or malignant tumor
• Papillary, atrophic or hyperplastic epithelium
• Candidal colonization of surface
• Heavy, chronic inflammatory infiltrate
Diagnosis
• Clinical appearance, location
Treatment
• Topical and/or brief course of systemic antifungal therapy
(optional)
• Observation
Prognosis
• Excellent
Osteomyelitis
Etiology
• An acute or chronic inflammatory process within the
medullary space or along the cortical surface of bone
• Usually due to extension of a periapical abscess
• Other common causes include physical trauma (fracture) or
bacteremia.
• Most common organisms include staphylococci and streptococci
• Pain, swelling, fever, lymphadenitis
• Sequestrum formation
• Lower lip paresthesia, occasionally with acute disease in
mandible
• Associated soft tissue swelling
• Acute phase may be unremarkable
• Ill-defined, patchy radiolucency (“moth eaten”)
Diagnosis
• Presentation and radiographic findings
• Microscopic evidence of intrabony inflammation, marrow
fibrosis, osteoclastic resorption, reduced osteoblastic activity,
nonviable bone
• Osteosarcoma
• Local extension of malignant tumor
• Osteoradionecrosis
Treatment
• Drainage and antibiotics for acute disease
• Débridement, sequestrectomy, antibiotics for chronic disease
• Reconstruction if necessary after disease is resolved
Prognosis
• Good
Osteoradionecrosis
Etiology
• A serious complication of tumoricidal doses of radiation to
the head and neck, usually > 60 Gy (6,000 rads)
• Radiation produces damage to the microvasculature, permit-
ting a hypoxic state, which, in turn, leads to a hypocellular
bony environment.
• Minor damage to the irradiated bone produces a nonhealing
wound, forming dead bone—necrosis.
• Usually affects the mandible
• Bone pain
• Exposed necrotic bone within radiation portal
• External fistula formation
• Pathologic fracture
• Irregular zones of mixed radiopacity and radiolucency
• Separation of nonvital bone (sequestrum) from remaining
viable bone
Diagnosis
• Radiographic and clinical features
• Biopsy results show nonvital bone.
• Locally recurrent tumor
• Osteomyelitis
• Osteosarcoma
• Radiation-induced sarcoma
Treatment
• After biopsy, débridement of bone preceded and followed by
hyperbaric oxygen therapy
• If necessary, resection and reconstruction
• Necessary tooth extraction and elimination of focal infection

within radiation portal 21 days prior to treatment
• Excellent preventive dental care
Prognosis
• Guarded
Periapical Granuloma
Etiology
• A mass of inflamed granulation tissue
• Forms secondary to pulp necrosis of the associated tooth
• May develop following periapical abscess formation or may
form as pulpal death eventuates without abscess precursor
• With acute exacerbation, pain and sensitivity develop.
• Tenderness at root apex on palpation
• Pain on biting or percussion of tooth
• Radiolucency at apex of tooth
• Size ranges up to 1 to 2 cm in diameter
• Root resorption not uncommon
Diagnosis
• Demonstration of nonvital pulpal component
• Periapical cemental dysplasia
• Periapical cyst
Treatment
• Conventional endodontic therapy
• Apical curettage/root end amputation if above measures fail
• Extraction of involved tooth
Prognosis
• Excellent
Sarcoidosis
Etiology
• Unknown; granulomatous disease process
• May represent a systemic response to a single provoking agent;
mycobacteria has been suggested but not proven
• Possible role of genetic factors coupled with disordered reac-
tions to foreign antigens
• Mucocutaneous
• Red to brown nodules/plaques with erythema nodosum
features
• Minor salivary glands of the lips and palate may be involved.
• Erythematous, hyperplastic gingiva
• Salivary/lacrimal
• Parotid, submandibular, and lacrimal glands may be
enlarged.
• Multiple organ systems, such as the following, may be involved:
• Particularly the lung, but also liver, endocrine glands, the
heart, and the reticuloendothelial and musculoskeletal
systems
• Heerfordt’s syndrome may be related to sarcoidosis (uveitis,
parotid gland enlargement, fever, cranial nerve palsies).
Diagnosis
• Demonstration of sarcoidal (noncaseating epitheloid) granulo-
mas in at least two organ systems
• Elevated serum angiotensin-converting enzyme levels are
usually present.
• Over 90% of cases have abnormal chest radiograph.
• Other causes of granulomatous inflammation must be ruled out.
• Tuberculosis
• Lymphoma (non-Hodgkin’s, Hodgkin’s)
Treatment
• Corticosteroids, if symptoms demand
• Severe or unresponsive cases: methotrexate
• Cutaneous lesions only: hydroxychloroquine
• Intralesional corticosteroids
Prognosis
• Generally good
Tooth Abnormalities
Abrasion
Etiology
• Excessive or abnormal wearing of teeth
• Commonly associated with use of smokeless tobacco, abrasive
dentifrices, cigars, and pipes, habitual grinding, improper
tooth-brushing techniques
• Pathologic wear of teeth associated with abnormal habits or
function or consumption of abrasive to coarse diets
• The so-called toothbrush abrasion at the gingival margin may
be related to abnormal incisal or occlusal forces producing
abfraction injury to enamel at the cementoenamel junction
• Causally related appearance includes the following:
• Incisal/occlusal wear related to habit or abrasive diet or
substance
• Cervical wear of posterior teeth with chronic, low-grade
toothbrush injury
• At occlusal and incisal surfaces, a generalized loss of crown
height
• At the cervical margin (cementoenamel junction), horizontal
V-shaped to saucerized notches (abfraction injury)
• With exposure of significant root surface, abrasion-related
injury, diffuse loss of cementum and dentin
• Pulp canals, containing tertiary dentin, are visible in advanced
cases.
Diagnosis
• Amelogenesis imperfecta
• Dentinogenesis imperfecta
Tooth Abnormalities 305
Treatment
• Restorative dental techniques
• Correction of habits, occlusal force discrepancies
Prognosis
• Good
Amelogenesis Imperfecta
Etiology
• Intrinsic enamel defect that affects all teeth of both dentitions
• Results from defective amelogenin genes on X and Y chromo-
somes and also chromosome 4 (tuftelin gene)
• At least 16 variants noted based upon inheritance pattern,
enamel qualities, and radiographic features
• Frequency of 1:14,000 to 1:16,000 of population
• One of three basic alterations of enamel may be seen: hypopla-
sia, hypomaturation, or hypocalcification
• Enamel hardness varies depending upon type of defect: normal
hardness in hypoplastic form but deficient amounts of enamel;
soft enamel in the hypocalcified variant but normal amounts of
enamel
• Color ranges from normal (hypoplastic) to dark yellow-brown
(hypocalcified)
• Radiographic changes range from normal density (hypoplastic)
to less dense (hypocalcified)
• May be noted in association with taurodontism (coronally
enlarged dental pulps)
• X-linked form demonstrates random vertical bands of normal
and hypoplastic enamel
Diagnosis
• Clinical and radiographic features
• Family history (autosomal, X-linked forms)
Treatment
• Full-crown restorations for esthetics
Attrition
Etiology
• Defined as a physiologic wearing of teeth secondary to normal
function/mastication
• Can involve all surfaces of teeth including interproximal,
incisal-occlusal, buccal, and lingual
• Abnormal occlusal-incisal relationship can predispose to accel-
erated rates of attrition
• Primary and permanent dentitions can be affected.
• Flattened occlusal surfaces and reduction of incisal height
• The loss of interproximal tooth surface (usually enamel only)
leads to gradual dental arch shortening.
• Asymptomatic
Diagnosis
• Generally proportional to age
Treatment
• Usually does not require specific management
• Elective restoration of occlusal/incisal surfaces to prevent over-
closure of jaws in function
Prognosis
• Excellent
Bulimia
Etiology
• A compulsive-eating disorder characterized by repeated
episodes of binge eating followed by vomiting or another form
of purging, including laxative abuse
• Cause may be biologic (neurometabolic disturbance), psycho-
logical (societal pressure for extreme thinness), or combined
(biopsychosocial)
• Nearly 1.2 million adolescent and young adult females are
affected in the United States; males are considerably less
affected. Up to 20% of college-age women are affected.
• Oral signs include erosion of teeth, gingivitis, xerostomia,
painless parotid gland enlargement, increased caries rate,
thermal hypersensitivity of teeth
• Specific patterns of enamel destruction (perimolysis) are
noted along the palatal and occlusal aspects of maxillary teeth,
sparing the buccal and labial surfaces.
• Mandibular teeth usually are affected less severely.
Diagnosis
• Recognition of oral signs
• Coordination of oral signs with other findings including der-
matologic signs: lanugo-like body hair, brittle hair and nails,
asteatotic skin, hand or finger calluses (related to self-induced
vomiting)
• Diet-induced enamel loss
• Chronic gastric reflux disease
Treatment
• Combined aggressive medical management, psychotherapy,
behavioral management, food intake management, and nutri-
tional counseling
Prognosis
• Fair to good
• Mortality (estimates range from 1–15%) is divided equally
between medical complications (electrolyte disturbance, acute
renal failure, cardiac complications) and suicide.
Dentinal Dysplasia
Etiology
• An inherited disorder (autosomal dominant) of circumpulpal
dentin with associated alterations of root morphology; no
other organs affected
• Premature tooth loss
• All teeth affected
• Two forms, as follows:
• More severe form (type I) characterized by “rootless teeth,”
with normal-colored crowns, obliterated pulp chambers,
multiple periapical radiolucencies (periapical granulomas/cysts)
• Less severe form (type II) characterized by amber-colored
primary teeth with susceptibility to wear; permanent teeth
of normal color with thistle-shaped pulp chambers; frequent
pulp stones noted
Diagnosis
• Combined clinical and radiographic features
• Normal clinical color of permanent teeth and periapical lesions
help to distinguish from dentinogenesis imperfecta
• Clinical crowns of primary teeth are amber and opalescent.
• Absent root formation (type I)
• Thistle-shaped pulp chambers and pulp stones (type II)
• Chemotherapy-/radiation therapy–induced root development
alteration
• Pulpal dysplasia
Treatment
• Teeth usually unsalvageable (type I)
• Observation
Prognosis
• Guarded
Dentinogenesis Imperfecta
Etiology
• Hereditary disorder (autosomal dominant) of dentin (1:8,000
frequency in population)
• May be seen in association with osteogenesis imperfecta
• Altered dentin matrix is related to the defective degradation of
dentin phosphoprotein during dentinogenesis.
• Primary and permanent dentition exhibit gray to brownish
opalescence
• Normal enamel fractures easily from defective underlying
dentin
• Severe tooth abrasion related to exposed dentin following
enamel loss
• Radiographically, roots are slender to spiked with pronounced
cervical constriction and obliterative pulpal calcification
• Constricted tooth cervix gives molar crowns a “tulip” profile
Diagnosis
• Clinical and radiographic appearance
• Family history
• Osteogenesis imperfecta
Treatment
• Functional and esthetic restorations (full crowns)
Erosion
Etiology
• Acid dissolution of enamel and dentin
• Loss of enamel and, less commonly, dentin secondary to chemical
(usually acids) action/demineralization
• Intrinsic sources relate to stomach acid presence within the
oral cavity.
• May be due to the following:
• Occupational exposure to acids
• Diet with acid exposure (phosphoric acid– containing
beverages; sucking on lemons)
• Chronic regurgitation/gastroesophageal reflux
• Bulimia-related vomiting
• Loss of enamel initially along lingual surfaces of anterior teeth
(bulimia, reflux)
• Labial enamel loss (beverage related, occupation related)
• Cupped dentin noted to occur more rapidly than adjacent
enamel loss on occlusal surfaces
• Existing metallic restorations (inlays, amalgam fillings) and any
protected enamel may be above the surrounding dentin, creating
a “ledge” effect.
• Smooth to polished appearance of maxillary incisors in chronic,
high-volume consumers of beverages (phosphoric or citric
acid–containing)
Diagnosis
• Correlation of appearance with diet, habits, environmental
exposure, underlying eating disorder, or chronic acidic reflux
• Factitial injury
Treatment
• Identification and elimination of cause
• Treatment of underlying etiology
• Dental restorative treatment subsequent to complete functional

evaluation, vertical dimension, and esthetics
Prognosis
• Excellent
Fluorosis: Chronic Endemic

Etiology
• Excessive dietary levels of fluoride: greater than one part per
million in drinking water (can lead to fluorosis in a dose-
dependent relationship)
• Childhood ingestion of fluoride dentifrice on a chronic basis
• Tooth enamel hypomaturation resulting from prolonged
ingestion of abnormally high levels of fluoride during tooth
development, usually between 2 and 3 years of age
• Enamel alterations ranging from local pitting to white opacity
or deeper brown mottling
• Distribution is symmetric and bilateral and occurs in all quad-
rants of the jaws.
Diagnosis
• Characteristic appearance and distribution
• Data concerning fluoride concentration in drinking water
should be obtained.
• Tetracycline-associated staining
Treatment
• Restorative dental treatment
• Cosmetic bleaching
Prognosis
• Excellent
Fusion
Etiology
• Merging of two tooth germs to create a single tooth
• A single large tooth (macrodont) is noted.
• One less tooth will be present in the dental arch.
• Common or separate pulp canals and roots
Diagnosis
• Radiographic evaluation
• One less tooth present in dental arch
Treatment
• If esthetics demand, removal and replacement
Prognosis
• Not applicable
Natal Teeth
Etiology
• Usually indicates prematurely erupted deciduous teeth
• Erupted teeth at birth
• Almost always are incisors
• 85% appear in mandible
Treatment
• If mobile, extraction
• Possible retention for functional, esthetic reasons
Malignant Nonodontogenic
Tumors
Ewing’s Sarcoma
Etiology
• Unknown
• Chromosomal translocations t(11;22), t(7;22), t(7;21) noted
• Gene rearrangement often noted, that is, (22;q12) and expres-
sion of the MIC2 gene
• Genetically related to primitive peripheral neuroectodermal
tumor via translocations t(11;22), (q24;q12)
• 60% in males; over 95% in those under 20 years of age
• Chiefly in bone and soft tissues
• Highly malignant
• Pain, numbness, and swelling often early complaints
• Diffuse, irregular, lytic bone lesion
• Cortical expansion variable
• Second most common bone tumor of children/adolescents
• Soft tissues of head and neck account for 11% of extraskeletal
sites
Diagnosis
• Radiographs often show “moth-eaten” appearance and lami-
nar periosteal bone reaction
• Cortex may be eroded or expanded
• Osteosarcoma
• Lymphoma
• Peripheral neuroectodermal tumor of bone
• Primitive rhabdomyosarcoma
• Neuroectodermal tumor of infancy
Malignant Nonodontogenic Tumors 325
Treatment
• Radiation and multiagent chemotherapy
Prognosis
• 54 to 74% 5-year survival rate in localized osseous form
• Late relapse not uncommon
Metastatic Cancer
Etiology
• Spread of a primary malignancy to the oral cavity structures or
jaws (usually from lung, breast, prostate, colon, kidney)
• Accounts for < 1% of oral malignancies
• Usually manifests in the jaws with pain and swelling
• Not uncommon is loosening of teeth or pathologic jaw fracture
• Soft tissue location is rare.
• Most frequent sites of primary neoplasms are kidney, lung,
breast, colon, prostate, stomach
• Intraosseous lesions with lytic, ill-defined radiolucencies
• As with the primary tumor
• Tumor marker studies (immunohistochemical) may be necessary
to define the site of origin.
Diagnosis
• Biopsy
• Primary soft tissue tumor
• Primary osseous tumor
• Periodontitis (localized)
Treatment
• Local radiation
• Combination chemoradiotherapy
Prognosis
• Poor
Osteosarcoma
Etiology
• May be associated with pre-existing bone disease such as the
following:
• Paget’s disease (10 to 15%)
• Fibrous dysplasia (0.5%)
• Mutation/amplification of p53, c-myc, c-JUN, c-fos, MOM2,
CDK4, SAS
• May present with pain paresthesia, trismus, nasal or paranasal
sinus obstruction
• May masquerade as an odontogenic infection
• Intraoral signs are as follows:
• Tooth mobility (vertical)
• Periapical radiolucency (teeth vital)
• Distal displacement of terminal molar
• Jaw mass may be ulcerated.
• Early intraoral findings
• Displacement of teeth
• Root resorption
• Absent or attenuated lamina dura
• Uniformly widened periodontal membrane space
• Later jaw bone findings
• Lytic, “moth-eaten” destruction
• Cortical destruction
• Soft tissue extension
• Erosion of mandibular canal
• 25% of cases have “sunburst effect” (radiating radiopaque
spicules)
• Sarcomatous stroma
• Osteoid production by neoplastic cells
• Four basic patterns (no prognostic significance) are as follows:
• Osteoblastic • Fibroblastic
• Chondroblastic • Telangiectatic
Diagnosis
• Correlation of clinical, radiographic, pathologic findings
• Fibro-osseous lesion
• Osteomyelitis
• Other form of sarcoma
Treatment
• Radical ablative surgery
• Hemimandibulectomy
• Partial maxillectomy ± orbital exenteration
• Adjuvant chemotherapy/radiotherapy
Prognosis
• Survival ranges from 12 to 58% at 5 years
• Mandibular lesions are associated with a greater survival rate
than are maxillary lesions.
Metabolic and Genetic Disorders
Amyloidosis
Etiology
• May be primary (idiopathic), secondary to systemic disease, or
familial
• Formation of a fibrillar protein deposited in soft tissues and
visceral organs with associated levels of dysfunction
• The primary form may produce obvious tongue enlargement
(macroglossia) and associated purpura, or nodular submucosal
alterations.
• The secondary form may be subtle; gingival tissues may contain
deposits of amyloid.
Diagnosis
• Appearance of tongue
• Systemic complaints
• Biopsy results: demonstration of amyloid deposits in tissues
(tongue, gingiva)
• Hyalinosis cutis et mucosae (lipoid proteinosis)
• Leukemic infiltrate
• Lymphangioma
• Neurofibromatosis
• Hemodialysis-related disorder
Treatment
• Directed to underlying cause (secondary)
• Localized amyloid tumors may be excised.
• Generally symptom related (dialysis, digitalis, depending upon
organ involvement)
Prognosis
• When renal impairment exists, transplantation may be necessary.
Metabolic and Genetic Disorders 331
Cherubism
Etiology
• Autosomal-dominant, fibroblast/giant cell–containing
condition
• May be secondary to somatic mutation, mapping
to chromosome 4p16.3
• No associated metabolic or biochemical alterations noted
• Possible linkage/association with Noonan’s syndrome
• Early signs in childhood
• Bilateral, symmetric enlargement of mandible
• Maxillary involvement less common and less prominent
• Dental arch/occlusal discrepancies may be noted.
• Unerupted teeth often noted
• Facial features include lower-third fullness and scleral
exposure at a forward resting gaze.
• Symmetric, multiloculated, expansile radiolucencies of
mandibular body and ramus
• Impacted/displaced teeth common
• Thinned cortices with scalloped medullary margins
• Older patients may exhibit maturation with bone fill in some
areas but with preservation of expanded bony profile.
Diagnosis
• Central giant cell granuloma (multiple)
• Langerhans cell disease (histiocytosis X)
• Hyperparathyroidism
• Multiple odontogenic keratocysts
Treatment
• Variable, ranging from cosmetic recontouring to local curettage
early in lesion development
• Active surgical intervention should be deferred until after the
pubertal growth spurt, if possible.
Prognosis
• Stability usually noted by end of skeletal growth
• Often regresses into adulthood, but variably so
Cleidocranial Dysplasia
Etiology
• Autosomal-dominant trait with high penetrance and variable
expressivity
• Mutations in SH3-binding protein on chromosome 4p16.3
• Widespread membranous and endochondral defects in cranio-
facial complex
• Chief head and neck manifestations include the following:
• Defective ossification
• Wormian bones with calvarial defects
• Delayed fontanelle and suture closure
• Variably developed clavicles often a prominent skeletal finding
• Long, narrow neck with variably drooped shoulders
• Midface deficiency secondary to hypoplasia of facial bones
and paranasal sinuses
• Ocular hypertelorism
• Palate with narrow, high-arched quality
• Delayed closure of mandibular symphysis
• Multiple unerupted and malpositioned teeth with lack of
cellular cementum
• Multiple supernumerary teeth
Diagnosis
• Radiographic findings (skull, jaw, chest)
• Achondroplasia
• Pyknodysostosis
• Hydrocephalus
Treatment
• For dental abnormalities, treatment options are as follows:
• Early orthodontic intervention
• Surgical exposure of unerupted teeth
• Extraction of supernumerary teeth
• Surgical correction of jaw deformities

• Dental reconstruction
Prognosis
• Stability with growth cessation
• Dental and oral rehabilitation can proceed as per usual after
surgery (see above) is completed.
Hyperparathyroidism
Etiology
• Primary form usually due to parathyroid adenoma
• Secondary form related to altered renal vitamin D metabolism
with secondary hypocalcemia
• Excessive parathormone secretion common to all forms
• Classic triad in patients over 60 years includes the following:
• Renal calculi/nephrolithiasis
• Subperiosteal resorption of phalanges
• Lethargy, psychotic-like state
• Fibrous/lytic bone lesions; chief oral finding is well-defined,
cyst-like radiolucencies of jaw(s)
• Osteoporotic bony changes
• Loss of lamina dura
• Duodenal ulcer formation
Diagnosis
• Increased serum calcium levels (primary)
• Increased urinary hydroxyproline levels
• Elevated serum parathormone
• Radiographic changes (phalanges, jaws)
• Histologic findings: identical to central giant cell granuloma
of jaws
• Cherubism
• Renal disease (osteodystrophy)
• Paget’s disease of bone (skull)—early stages
• Multiple odontogenic keratocysts (nevoid basal cell carcinoma
syndrome)
Treatment
• Primary hyperparathyroidism: removal of abnormal gland(s)
• Secondary hyperparathyroidism: management of renal disease
Prognosis
• Good
Therapeutics
Actinomycosis
• Systemic therapy: penicillin or tetracycline in large doses for
3–6 mo
• Wide excision of infected tissue
Acute Herpetic Gingivostomatitis

• Systemic therapy
• Valacyclovir 500 mg #20; 1 tablet twice daily × 10 d
• Acyclovir 400 mg #50; 1 tablet 5 times daily × 10 d
• Fluids
• Analgesia
Acute Necrotizing Ulcerative Gingivitis

• Débridement of necrotic tissue
• Aggressive oral hygiene and plaque control
• Metronidazole 250 mg #40; 1 4 times daily × 10 d
Angioedema
• Antihistamine: diphenhydramine 50 mg capsules #12;
1 every 6 h × 2–3 d
• Doxepin 25 mg tablets #12; 1 every 6 h × 2–3 d
• Prednisone 10 mg tablets #12; 4 tablets daily × 3 d
Aphthous Stomatitis
• See “Recurrent Aphthous Stomatitis.”
Behçet’s Disease
• Treat as for aphthosis (see “Recurrent Aphthous Stomatitis”).
• Refer to a dermatologist, a rheumatologist, or an ophthalmolo-
gist, depending on organ involvement, for ongoing care,
which may include systemic immunosuppressive and/or
anti-inflammatory drugs.
Candidiasis
• Identify and correct provocative factors.
• Topical therapy
• Nystatin oral suspension (100,000 units/mL); rinse 5 mL
and swallow 4 times/d
• Clotrimazole (Lotrimin) solution 1%; rinse 5 mL and
swallow 4 times/d
Therapeutics 339
• Clotrimazole troches (Mycelex) 10 mg; dissolve 1 troche in

mouth 5 times/d
• Clotrimazole vaginal tablets 1/ 2 of 500 mg tablet dissolved
in mouth bid
• Fluconazole (Diflucan) 100 mg #15; 2 tablets on the first day,
1 tablet days 2–7, 1 tablet every other day for days 8–21
• Ketoconazole (Nizoral) 200 mg #21; 1 tablet every day with
breakfast × 21 d
• Itraconazole (Sporanox) 200 mg #21; 1 tablet every day
with breakfast × 21 d
• May use shorter duration for less severe infections
Cheilitis Glandularis
• Challenging to treat
• Trials of therapy
• Intralesional corticosteroids as triamcinolone acetonide
5–10 mg/mL; inject 1–3 mL per session with sessions at
3–4 wk intervals
• Systemic antibiotic: tetracycline 500 mg tid
• Systemic corticosteroid: prednisone 5 mg tablets #40
– Take each morning for 8 with breakfast, 8-8-6-6-4-4-
2-2 mg, stop
– Will shorten the course of an individual episode but not
change the natural history of the disease
Cheilitis Granulomatosa
• Intralesional corticosteroids such as triamcinolone acetonide
3–4 wk intervals
• Systemic corticosteroids: prednisone 5 mg tablets #40
– Take each morning for 8 d with breakfast, 8-8-6-6-4-4-
2-2 mg-stop
– Will shorten the course of an individual episode but not
change the natural history of the disease
• Dapsone 25 mg tablets
– Check baseline complete blood count (CBC), liver function
tests, urinalysis, and glucose-6-phosphate red blood cell
enzyme level before treatment.
– Take each morning with breakfast, 1 × 3 d, 2 × 3 d,

3 × 3 d, 4 × 7 d, and 5 daily thereafter
– Check CBC and liver function every month for 3 mo,
then every 3 mo thereafter.
– Use for long-term control of disease
Crohn’s Disease
• Intralesional corticosteroids such as triamcinolone acetonide
3–4 wk intervals
• Systemic corticosteroid: prednisone 5 mg tablets #80
– Take each morning with breakfast for 16 d as 8/d × 4 d,
6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop
– Will reduce disease activity as topical corticosteroids or
systemic nonsteroidal anti-inflammatory drugs (NSAIDs)
are started
– Check baseline CBC, liver function tests, urinalysis, and
glucose-6-phosphate red blood cell enzyme level before
treatment.
3 × 3 d, 4 × 7 d, and 5 daily thereafter
Drug-Induced Stomatitis (Stomatitis Medicamentosa)
• Topical therapy (compounded rinses)
• Option 1
– Diphenhydramine 200 mg, viscous lidocaine 90 mL,
Maalox suspension 90 mL, distilled water 180 mL
– Swish 5 mL for 2 min and expectorate 3–4 times/d.
• Option 2
– Dexamethasone 100 mg, viscous lidocaine 60 mL, diphen
hydramine 200 mg, sorbitol 15 mL, Maalox suspension to
275 mL
Therapeutics 341
• Systemic therapy: prednisone 5 mg tablets #80

• Take each morning with breakfast for 16 d as 8/d × 4 d,
6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop
• Will reduce disease activity as topical corticosteroids or
systemic NSAIDs are started
Erythema Multiforme
• Option 1
• Option 2
– Dexamethasone 100 mg, viscous lidocaine 60 mL,
diphenhydramine 200 mg, sorbitol 15 mL, Maalox
suspension to 275 mL
• Prednisone 5 mg tablets #80
6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop
• Acyclovir 200 mg tablets #42 (if triggered by herpes simplex
virus infection); 1 tablet every 4 h for 7 d or 1 tablet bid-tid
as prophylaxis
Exfoliative Cheilitis
• Identify possible topical or drug-related causative agents
(eg, gold, toothpaste, mouthwash, lipstick).
• Determine if factitial cause(s) is present.
• Topical therapy: see “Candidiasis”
• Systemic therapy: see “Candidiasis”
Fissured Tongue
• Brush tongue surface 10–15 times with dentifrice after meals
and at bedtime to remove debris that causes halitosis.
Geographic Tongue
• Topical therapy
• Fluocinonide gel/cream 0.05% 60 g; apply after meals and at
bedtime
• Clotrimazole troches (Mycelex) 10 mg; dissolve 1 troche in
mouth 5 times/d
• Clotrimazole vaginal tablets 1/ 2 of 500 mg tablet dissolved in
mouth bid
• Tacrolimus (Protopic) ointment 0.1% 60 g; apply after meals
and at bedtime
Hairy Tongue
• Topical therapy: dilute H2O2 (1 part 3% H2O2:1 part H2O);
brush tongue after meals and at bedtime for black hairy tongue
Hand-Foot-and-Mouth Disease
• Fluids
• Analgesia
• Recovery expected quickly
Herpangina
• Fluids
• Analgesia
• Recovery expected quickly
Herpes Zoster
• Topical therapy
• Calamine lotion for wet, oozing cutaneous lesions
• Doxepin (Zonalon) cream for pain relief of acute lesions
• Acyclovir 400 mg tablets #100; 2 tablets 5 times daily × 7–10 d
• Famciclovir 500 mg tablets #21; 1 tablet 3 times daily × 7 d
• Valacyclovir 500 mg tablets #42; 2 tablets 3 times daily × 7 d
Impetigo
• Topical therapy: mupirocin ointment applied twice daily
• Penicillin V potassium 250 mg tablets #40; 1 tablet 4 times
daily × 10 d
• Erythromycin base 250 mg tablets #40; 1 tablet 4 times daily
× 10 d
• Dicloxacillin 250 mg tablets #40; 1 tablet 4 times daily × 10 d
Therapeutics 343
Lichen Planus
• Topical therapy
• Betamethasone cream (0.1%) 60 g; apply after meals and at
bedtime
• Fluocinonide gel/cream 0.05% 60 g; apply after meals and
at bedtime
• Tacrolimus (Protopic) ointment 0.1% 30 g; apply after
meals 3 times dailyand at bedtime, do not eat or drink for
30 min; taper frequency depending on response
• Intralesional therapy: triamcinolone acetonide 5–10 mg/mL;
inject 1–3 mL per session with sessions at 3–4 wk intervals
6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop
– Check baseline CBC, liver function tests, urinalysis, and
glucose-6-phosphate dehydrogenase enzyme level before
treatment.
3 × 3 d, 4 × 7 d, and 5 × daily thereafter
– Check CBC and liver function every month for 3 mos,
– Use for long-term control of disease.
• Hydroxychloroquine (Plaquenil) 250 mg #100; 2 tablets
with breakfast for 4 wk, then 1 tablet daily for maintenance
– Baseline ophthalmology consultation; repeat every 6 mo
to monitor for retinal toxicity
Lupus Erythematosus
• Topical therapy
• Fluocinonide gel/cream 0.05% 60 g; apply after meals and
at bedtime
• Tacrolimus (Protopic) ointment 0.1% 30 g; apply after
meals 3 times daily, do not eat or drink for 30 min
• Intralesional therapy: triamcinolone acetonide 5–10 mg/mL;
inject 1–3 mL per session with sessions at 3–4 wk intervals
Melkersson-Rosenthal Syndrome
• See “Fissured Tongue.”
• Orofacial granulomatosis—see “Cheilitis Granulomatosa”
Nevus
• All pigmented nevi should be excised, if reasonable from a
surgical point of view.
Pemphigoid
• Refer to a dermatologist or an ophthalmologist, depending on
organ involvement, for ongoing care, which may include sys-
temic immunosuppressive and/or anti-inflammatory drugs.
• For localized oral pemphigoid/gingival pemphigoid, apply
topical therapy: fluocinonide 0.05% gel/cream 60 g
• Apply to early lesions after meals and at bedtime.
• Do not apply to ulcers.
• May be used for 1–2 h with mouthguard for occlusive therapy
• Systemic therapy for severe, chronic disease
6/d × 4 d, 4/d × 4 d, 2/d × 4 d, stop
– Check baseline CBC, liver function tests, urinalysis and
glucose-6-phosphate dehydrogenase enzyme level before
treatment.
– Check CBC and liver function every month for 3 mo, then
every 3 mo thereafter.
• Tetracycline and niacinamide
– 500 mg of each administered tid
Pemphigus Vulgaris
• Coordinate overall management with patient’s internist/primary
care physician since treatment of this disease requires systemic
immunosuppression and/or use of anti-inflammatory drugs.
• Management of oral lesions will consist of systemic immuno-
suppressive agents.
Therapeutics 345
• Local/intralesional therapy may be a useful adjunct following

an initial good measurable response to systemic glucocortico-
steroid dosing.
• Systemic therapy: prednisone 10 mg tablets #150
• Take each morning with breakfast at a total daily dose of
1 mg/kg of body weight.
• Taper slowly over several months as clinical response
permits to maintenance dosing.
• Management of prednisone side effects is important.
• Corticosteroid-sparing systemic therapy
• Azathioprine 1–3 mg/kg; dosing spaced morning and evening
• Mycophenolate mofetil 500 mg tablets; 1.5 g bid
• Severe or unresponsive disease
• Plasmapheresis
• Pulse cyclophosphamide (Cytoxan) IV for 3 wk
– Monitor response.
– Continue on orally administered immunosuppressants.
• IVIg therapy
• Local therapy for focal residual lesions: intralesional
triamcinolone suspension 10 mg/mL
Plasma Cell Gingivitis

• Identify contact allergen(s) and avoid exposure.
• Topical therapy: fluocinonide gel/cream 0.05% 60 g; apply
after meals and at bedtime
• Systemic therapy: griseofulvin 250 mg tablets #150; take 1
with each meal for 7 wk
Pyostomatitis Vegetans
• Seek the underlying inflammatory bowel disease.
• See “Crohn’s Disease.”
Radiation-Induced Mucositis
• Topical therapy
• Benzydamine rinses
• Saline/bicarbonate rinses 2.5 mL each in 125 mL water;
5 mL rinsed bid
• Chlorhexidine 0.12% compounded as alcohol-free formula
– Store in a light-protective container.
– 15–30 mL rinsed bid
• See “Drug-Induced Stomatitis.”
• Systemic therapy: analgesics prn
Recurrent Aphthous Stomatitis (Aphthosis)

• Classify disease into simple versus complex
• Simple aphthosis
• Amlexanox paste 5 g (Aphthasol); apply to ulcers after
meals and at bedtime
• Fluocinonide 0.05% gel/cream 60 g
– Apply to early lesions after meals and at bedtime.
– Do not apply to ulcers.
• Compounded rinse option 1
– Diphenhydramine parenteral (or 12.5 mg/5 mL non-
alcoholic elixer) 200 mg, viscous lidocaine 90 mL,
Rinse 5 mL—expectorate 4–6 times daily.
• Compounded rinse option 2
Dexamethasone (10 mg/mL) 10 mL, diphenhydramine
200 mg, viscous lidocaine 60 mL, Maalox suspension 85
to 275 mL
Rinse 5 mL—expectorate 3–5 times daily.
• Complex aphthosis
• Laboratory evaluation for “correctable causes”: CBC, red
blood cell folate, serum ferritin, serum vitamin B12, serum
iron studies, serum zinc
• Topical therapy as for simple aphthosis
• Systemic therapy for severe, painful, chronic complex
aphthosis
– Prednisone 5 mg tablets #40
– Take each morning with breakfast for 8 d 8-8-6-6-4-4-
2-2 mg, stop
– Will shorten the course of an individual episode but
not change the natural history of the disease
– Colchicine 0.5 mg tablets
– Take 1 each morning with breakfast for 1 wk; if
tolerated, increase to 2 tablets each morning
– May suppress disease activity
– Pentoxifylline (Trental) 400 mg tablets; 1 tablet
3 times/d with meals
– Dapsone 25 mg tablets
– Check baseline CBC, liver function tests, urinalysis
and glucose-6-phosphate dehydrogenase enzyme level
before treatment.
Therapeutics 347

– Use for long-term control of disease.
Recurrent Herpes Simplex Labialis or Stomatitis

• Topical therapy
• Penciclovir cream (Denavir) 1% 1.5 g tube; apply at the
onset of symptoms every 2 h × 4 d
• Docosanol cream (Abreva) 10%; apply topically at the onset of
symptoms q2–3h 5 times daily
• Acyclovir ointment 5% 3 g tube; apply at the onset of
symptoms 6 times daily × 7 d
• Acyclovir 200 mg tablets #35
– 1 tablet 5 times daily × 7 d
– Start medication with premonitory symptoms to shorten
the course of the episode.
• Acyclovir 200 mg tablets
– 3 tablets daily to prevent reactivation in bone
marrow transplant recipients
Sjögren’s Syndrome
• Topical therapy
• Moisten mouth with cool water or ice chips.
• Avoid alcohol-containing mouth rinses.
• Avoid drugs that produce xerostomia.
• Limit caffeine intake.
• Use Vaseline on lips at night (a thin coating).
• Drink milk with meals
• Saliva substitutes
• Liquid, tablet, or gel forms
• Available over the counter
• Pilocarpine (Salagen) 5 mg tablets #100; take 1 tablet
3 times daily
• Cevimeline capsules (Evoxac) 30 mg capsules #100; take
1 capsule 3 times daily
Stevens-Johnson Syndrome
• Option 1
– Swish 5 mL for 2 min and expectorate 3–4 times/d
• Option 2
– Dexamethasone 100 mg, viscous lidocaine 60 mL,
diphenhydramine 200 mg, sorbitol 15 mL, Maalox
suspension to 275 mL
– Swish 5 mL for 2 min and expectorate 3–4 times/d
6/d × 4 d, 4/d × 4 d, 2/d × 2 d, stop
• Acyclovir 200 mg tablets #42 (if triggered by herpes simplex
virus infection); 1 tablet every 4 h for 7 d or 1 tablet bid-tid
as prophylaxis
Tuberculosis
• Systemic therapy (prolonged treatment with at least 2 drugs)
• Isoniazid 300 mg daily × 6 mo
• Rifampin 450–600 mg daily × 6 mo
• Ethambutol 15 mg/kg daily for first 2 mo
• Pyrazinamide 1.5–2.5 mg/kg for first 2 mo
Wegener’s Granulomatosis
• Sulfamethoxazole/trimethoprim (Bactrim DS) Septra DS
1 twice daily
• Prednisone 1 mg/kg daily
• Cyclophosphamide
Zoster
• See “Herpes Zoster.”
349
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Diagnosis of Oral Diseases

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2 PDQ ORAL DISEASE

Actinic (Solar) Cheilitis

• Possibly photodynamic therapy

Lichenoid Drug Eruptions

Treatment of Oral Lichen Planus

Proliferative Verrucous Leukoplakia

Smokeless Tobacco Keratosis (Snuff Pouch)

Both photographs courtesy of Dr. John S. Greenspan.

White Sponge Nevus

Hereditary Hemorrhagic Telangiectasia

Treatment of AIDS-Associated Form

Plasma Cell Gingivitis

Herpetic Stomatitis: Primary

Mucous Membrane Pemphigoid

Recurrent Herpetic Stomatitis: Secondary

Varicella and Herpes Zoster

Acute Necrotizing Ulcerative Gingivitis

• Rinses of chlorhexidine, topical povidone-iodine

• May persist for several weeks to months

• Other immunomodulating drugs may be helpful (dapsone,

• Management of any associated malabsorption may be helpful.

Photograph courtesy of Dr. John Knapp.

Squamous Cell Carcinoma

• Serologic studies (eg, VDRL [Venereal Disease Research

Differential Diagnosis: Clinical

Differential Diagnosis: Microscopic

Mucosal Malignant Melanoma

Mucosal Melanotic Macule and Ephelides

Mucosal Pigmentation: Extrinsic

Pigmentation Disorders: Drug Induced

Drugs Capable of Producing Tissue Pigmentation

Pigmentation Disorders: Physiologic

Pigmentation Disorders: Smoker’s Melanosis

Focal Epithelial Hyperplasia

Papillary Hyperplasia (Palatal Papillomatosis)

Verruca Vulgaris (Oral Warts)

Connective Tissue Lesions

Fibrous Hyperplasia: Denture-Related

Florid Osseous Dysplasia

Giant Cell Granuloma

Gingival Hyperplasia: Generalized

Granular Cell Tumor (Granular Cell Myoblastoma)

Lingual Bone Defect (Stafne Bone Cyst;

Clinical Presentation of MEN III

Laboratory Findings of MEN III

Microscopic Findings of Mucosal Neuroma

Radiographic Findings (When Intrabony Lesions Are Present)

Prognosis and Complications

Periapical Cemento-osseous Dysplasia

Torus: Palatal and Mandibular

Salivary Gland Diseases

Mucus Retention Cyst

ing pyknotic cellular debris represent the “stars” in the so-

Differential Diagnosis: Clinical

Differential Diagnosis: Microscopic

• Soft tissue tumor (primary)

Aneurysmal Bone Cyst

Calcifying Odontogenic Cyst

Differential Diagnosis: Radiographic

Dental Lamina Cyst (Bohn’s Nodules;

Differential Diagnosis: Radiographic

Glandular Odontogenic Cyst

Lateral Periodontal Cyst